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Abstract

Ganglioneuromas are benign neoplasms of the neural crest, occurring rarely in the adrenal glands. This study presents our experience regarding diagnostic and therapeutic management of these neoplasms and a review of the relevant literature. Among 150 patients with of incidentalomas, we had 7 primary ganglioneuromas. Their clinical, imaging, and operative data were collected retrospectively, and the literature was reviewed using MEDLINE. There were 4 females and 3 males, with mean age of 50 years (range, 39-64). All neoplasms were discovered incidentally with ultrasonography and were evaluated subsequently with computed tomography (CT). One patient was studied further with (131)I-MIBG due to asymptomatic increased in urine vanillylmandelic acid, and 1 patient with history of breast cancer underwent additional FDG-PET/CT. All but 2 patients were asymptomatic. Two patients complained of epigastric pain and hypertension, respectively. The preoperative mean size on CT was 6.8 cm, whereas the postoperative true mean histologic size was 7.7 cm. Both patients who were evaluated with radionuclide studies had false positive results, suggestive of pheochromocytoma and adrenal metastasis, respectively. Three patients underwent open adrenalectomy due to preoperative suspicion of carcinoma, and the remaining 4 underwent laparoscopic anterior adrenalectomy. Histologically, all 7 neoplasms were completely differentiated, mature ganglioneuromas. We had no mortality or significant morbidity. No recurrence occurred during a mean follow-up of 6 years (range, 1-18). Adrenal ganglioneuromas are rare incidentalomas that can mimic primary or secondary adrenal malignancies as well as pheochromocytomas. Despite their usually large size, resection via laparoscopic approach is safe and effective.

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... The most common location is the posterior mediastinum (41.5%) followed by the retroperitoneal space (37.5%) including the adrenal glands (21%), other less common locations are in the neck (8%) where few case reports of a retropharyngeal ganglioneuroma where reported [3,4]. ...
... Ganglioneuroma is a benign neoplasm originating from ganglion cells of the sympathetic chain along with other tumors including neuroblastomas and ganglioneuroblastomas, where they compose of variable degrees of immaturity and malignant behavior [3]. ...
... The most common location of ganglioneuroma is the mediastinum followed by the aortocaval sympathetic ganglia within the retroperitoneal space. Less frequently, ganglioneuroma can arise in the adrenals, and affect primarily pediatric patients and young adults [3,6]. ...
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BACKGROUND Ganglioneuroma is a rare benign sympathetic chain neoplasm which rarely arise from the adrenal gland in adults and exceedingly rare for ganglioneuroma to arise from different origins in the same patient. We present a rare case of retroperitoneal ganglioneuroma concurring with an adrenal ganglioneuroma in an adult patient. CASE REPORT A 17-year-old healthy male underwent computed tomography (CT) scan after road traffic accident where he was found to have a left adrenal homogeneous hypovascular lesion with another retroperitoneal lesion seen within the right para-colic gutter with similar radiological features. Successful surgical resection was obtained, and histological diagnosis of both lesions was benign ganglioneuroma. CONCLUSIONS Adrenal and extra-adrenal ganglioneuroma can, although rare, concur together. Key findings are the similar radiological appearance of the lesions with typical hypovascular progressive pattern of enhancement. Appropriate pre-surgical diagnosis can aid to proper management since ganglioneuroma needs wide surgical resection due to its high chance of recurrence.
... Most previous studies dealt with heterogeneous groups of ganglioneuroma and did not focus on AGN. Only a few original articles and several additional case reports on AGN have been published so far [8][9][10][11]. Symptoms related to hormonal abnormalities, major surgery-related complications, mortalities, and recurrences were rarely reported. ...
... According to other case series on AGN, asymptomatic patients account for 62.9-93.3 % of cases [9][10][11]. In our study, 29 (82.9 %) patients were asymptomatic and the rest showed symptoms related to mass effect. ...
... Open and laparoscopic operations were chosen based on the surgeon's preference, and tumor size was not a statistical significance factor in the decision. However, surgeons should note that preoperative findings are apt to underestimate tumor size [8][9][10][11]. Our preoperative tumor size was 5.3 ± 2.7 cm, whereas pathologic reports revealed a size of 6.3 ± 3.3 cm. Even though there was no statistically significant difference between the two, this should be taken into consideration by the surgeons. ...
Article
Background: Ganglioneuromas are benign tumors that rarely develop from adrenal glands. In this study, we present our clinical experience of patients with adrenal ganglioneuroma (AGN). Methods: Demographic, diagnostic, surgical, and pathologic findings of patients who were adrenalectomized as a result of AGN were retrospectively reviewed from the database of a tertiary referral hospital. Results: Among 1784 patients who underwent an adrenalectomy between 2002 and 2015, 35 (1.9 %; 14 males, 21 females) were diagnosed with AGN. Mean age was 33.4 ± 18.7 years (0-84). Twenty-nine (82.9 %) were asymptomatic, four (11.4 %) complained of abdominal discomfort, and two (5.7 %) had abdominal distension. Preoperative computed tomography (CT) reported AGN in 22 (62.9 %) cases. Precontrast Hounsfield units, increased postcontrast phase attenuation, and well-defined borders were characteristic CT features of AGN. Mean tumor size was 6.3 ± 3.3 cm (range, 1.5-16.0). No recurrence occurred during a median follow-up period of 19 months (range, 1-120). Conclusion: AGN was asymptomatic in most cases and diagnosis may be challenging. Adrenalectomy is a safe treatment modality for AGN and ensures favorable outcomes when diagnosed.
... In addition, it is generally believed that large adrenal tumours (>3cm) are usually functional and heterogeneous compared with small adrenal tumours. 12 This study aimed to retrospectively investigate the CT characteristics of AGNs and to determine whether there were any significant differences between large AGNs (>3 cm) and small AGNs ( 3 cm) with a relatively large sample size. ...
... It has been reported that adrenal tumours that are larger than 3 cm are likely to be functional, while those that are less than 3 cm are not. 12 In addition, tumour size is a crucial decisive factor for the prognosis of adrenal tumorus. 7 Hence, this current study analysed the AGNs based on their tumour size using a cut-off medium diameter of 3 cm. ...
... Previous reports demonstrated rates of calcification in AGNs of 30-60%. 12,22,27 Of the 30 patients, six large AGNs (42.9%) had calcifications on CT, but none was observed in the small AGNs (P <0.05). The presence of calcifications has been reported to be a characteristic feature for the diagnosis of AGNs. ...
Article
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Objective To investigate the computed tomography (CT) characteristics of adrenal ganglioneuromas (AGNs) and to determine the ability of CT scanning to distinguish between large (>3 cm) and small (≤3 cm) AGNs. Methods This retrospective study searched the electronic medical record system of a hospital between January 2008 and July 2019 in order to identify patients with pathologically-confirmed AGNs that underwent three phases of CT scanning. The CT features were compared between large and small AGNs. Results A total of 30 patients with pathologically-confirmed AGNs were included in the study. The majority of patients (76.7%; 23 of 30) were asymptomatic and there were nonspecific symptoms in seven patients. The ‘pointed peach’ sign appeared in more than half of the patients (53.3%; 16 of 30). The CT value of the arterial phase, progressive enhancement, morphology and calcification in the CT images were found to be significantly different between large and small AGNs. Progressive enhancement was more likely to occur in small AGNs. Most large AGNs had irregular shapes, while small AGNs were likely to be round or oval with a smooth border. Calcifications were noted in large AGNs (42.9%; six of 14). Conclusion CT scanning can show many of the key imaging characteristics of AGNs used to distinguish between large and small AGNs.
... GN is composed of mature ganglion cells and Schwann cells in a fibrous stroma. Adrenal GN is usually asymptomatic and hormonally inactive, but some of them secrete catecholamines and their metabolites -cortisol or androgens (1)(2)(3). ...
... Macroscopically, GNs are large encapsulated firm masses with homogeneous, grayish-white cut surface. On immunohistochemical staining, related symptoms and may be hormonally active, secreting catecholamine or its metabolites (1)(2)(3). ...
... references they are characterized with S-100, NSE, and synaptophysin positivity (1,3). The histology of adrenal GN has been reported to be overlap with that of cortical adenoma/pheochromacytoma, and myelolipoma (1). ...
Article
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We report a case of a dopamine-secreting giant primary adrenal ganglioneuroma (GN) in a 29-year-old male patient. Although the patient was clinically silent, the 24-hour urine levels of dopamine, normetanephrine, homovanillic acid and vanillyl mandelic acid were elevated. Abdominal ultrasonography and magnetic resonance imaging showed a large solid tumor with calcifications and a slightly lobular edge on the left adrenal gland. A tumor, 13 x 23 x 25 cm in size, was completely resected without morbidity. A 2-year follow-up with computed tomography showed that the postoperative course of the patient was uneventful.
... It may arise from base of skull, neck, posterior mediastinum, retroperitoneum or adrenal glands. 1 There is female predominance with peak age of incidence 10 to 40 years and most of the cases occur before the age of 20. 2 These tumors are slow-growing and are asymptomatic; sometimes diagnosed as incidental findings. They are usually nonfunctioning and give rise to symptoms only due to significant pressure on surrounding structures. 1 The important differentials are neuroblastoma and ganglioneuroblastoma. Nonetheless, biopsy is the only definitive tool of diagnosis. ...
... They are usually nonfunctioning and give rise to symptoms only due to significant pressure on surrounding structures. 1 The important differentials are neuroblastoma and ganglioneuroblastoma. Nonetheless, biopsy is the only definitive tool of diagnosis. ...
... 4 Increased MIBG uptake is found in 57% of GN and 92% of neuroblastoma. 3 High level of MIBG uptake along with increased urinary catecholamine levels can differentiate GN from neuroblastoma. 1 Nonetheless CT scan is the most frequent radiological investigation performed because it identifies the organ of origin, extent of tumor, vascular encasement, regional invasion, adenopathy and calcification if any. 5 Histological evidence is required to establish the diagnosis of GN and pre-operative diagnosis is usually difficult to establish. Tissue can be taken using ultrasound or CT guided needle aspiration or open biopsy. ...
Article
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Ganglioneuroma is a rare, benign, slow-growing asymptomatic tumor of the neural crest cells. It occurs in one per million population with female predominance. It most commonly occurs in the retro=peritoneum. Although CT scan and other imaging modalities can be used, its diagnosis is essentially based on histopathology. We report a case of 18 year old male who presented with abdominal pain and a mass in the left lumbar quadrant. CT scan abdomen showed a retroperitoneal mass encasing aorta, IVC and left common iliac artery. A diagnosis of Ganglioneuroma was established on ultrasound guided Trucut biopsy. He underwent laparotomy for total excision of mass. Per-operatively the mass was separated from the vital structures and the major blood vessels. His post-operative course was uneventful and he was discharged on 7th post-operative day. His final histopathology confirmed the initial diagnosis of ganglioneuroma. Nonetheless, a possibility of ganglioneuroma should be kept under consideration in cases of all retroperitoneal extra-adrenal masses.
... Most non-cranial GNs are located in the retroperitoneum and posterior mediastinum [2,3]. However, other rare sites of non-cranial GNs may include the adrenal gland, small intestine, colon, urinary bladder and para-testis [4][5][6][7]. ...
... It has been reported that the prevalence of adrenal incidentaloma is approximately 0.2, 3, and 7% in young patients, those past their fifth decade, and those past their seventh decade, respectively [4]. The differential diagnosis of adrenal incidentalomas may vary from a simple benign cyst or lipoma to adrenal carcinoma. ...
... GN is usually observed in 10 to 30-year olds [12], and several studies have shown that it may also be observed between the ages 40 and 50 [13,14]. Commonly reported locations include the posterior mediastinum, retroperitoneum, small intestine, colon and urinary bladder [4][5][6][7], while adrenal and para-testicular GN locations are very rare and are often unexpectedly discovered [9]. GN is usually silent and asymptomatic and becomes symptomatic due to hormone production or adjacent organ compression. ...
Article
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Background: Ganglioneuromas (GNs) are composed of mature ganglion cells and Schwann cells with a fibrous stroma; GNs are most often observed in children and young adults. The majority of non-cranial GNs are located in the retroperitoneum and posterior mediastinum. Other reported rare sites include the adrenal gland, small intestine, colon and urinary bladder. However, para-testicular GNs are even more rare. Case presentation: Herein, we report the case of a patient with concurrent adrenal GN and thyroid papillary carcinoma who developed paratesticular GN eighteen years later. Conclusions: We conclude that there is an association among papillary thyroid carcinoma, GN and MEN2 syndromes. This case report may provide important information for the proposed association. However, further studies are required.
... The most common site is mediastinum, retro peritoneum and adrenal glands. [1] [2] Ganglioneuromas of the adrenal gland are extremely rare. These tumors affect preferentially young people with the majority reported to occur before the age of 20. ...
... They are found incidentally while, investigating unrelated pathology. [1] It is rare to diagnose them, presenting with lump in abdomen and gross hematuria. We are reporting first such case. ...
... On USG, CT scan and MRI, they have different imaging characteristics. [1] [7] Modha et al. in their case series report, no patient was diagnosed preoperatively, in spite of imaging study in all cases and FNAC in three out of five cases. [8] The treatment for this condition is complete surgical resection through either an open or a laparoscopic approach. ...
Article
ARTICLE INFO ABSTRACT A Ganglioneuroma is the rarest and benign tumor of neural crest origin. It originates from the neural crest cells wherever sympathetic nervous tissue present, like in the retroperitonium, mediastinum, and adrenal medulla. They are common in children and young before the age of 20. Ganglioneuroma of adrenal gland is very rare. We are presenting rare case adrenal Ganglioneuroma in a five-year-old female child presented with large palpable left lion mass and intermittent hematuria. CT abdomen revealed, a 15x8cm-cm non-homogenous mass in the left adrenal gland compressing left kidney. Considering the size of tumor differential diagnosis of Neuroblastoma and other malignant adrenal tumor made. Patient underwent open lapromatomy for tumor removal. Histopathology examination revealed a well – encapsulated benign tumor of mature ganglion cells and Schwann-like cells arranged in fascicles.
... The most common GN locations are the posterior mediastinum (41.5 %), and the retroperitoneal space (37.5 %) -including the adrenal glands (21%), while less commonly the tumor appears in other locations -mostly in the neck region (8% ), e.g. the retropharyngeal space as in the case described by Yang et al. (1,2). Adrenal location of ganglioneuroma is rare: Linos and co-authors identified approximately 230 of such literature cases (3). ...
... Although the tumors are benign lesions, due to their size and radiological features, they can often be confused with malignancies. Unclear clinical and sometimes radiological presentations are the reasons for the higher than expected rate of open surgery of GNs (3,5,6). ...
... The average tumor size at the time of diagnosis is ca. 6-8 cm (3,5). In three of our five patients this size was reached. ...
Article
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Background. Ganglioneuroma (GN) is a rare, benign, neuroblastic tumor. It may arise from the sympathetic plexus or adrenal medulla. Its most common location is the posterior mediastinum (41.5%), while in approximately 21% of patients the tumor is located in the adrenal gland. Median age of adults at diagnosis is approximately 40 to50 years of age. Since GN is most commonly asymptomatic, the diagnosis is usually accidental, i.e. when radiological examinations are performed due to other indications. This slow-growing tumor can reach a large size. In a small percentage of patients the tumor secretes catecholamines. We present five ganglioneuroma patients, who were hospitalized in our Department from 1993 through 2012. Their mean age was 31 years. In four cases the tumor was located in the adrenal gland and in one - extra-adrenally, which was revealed during surgery. In three out of five presented cases pathological hormone secretion by the tumor was confirmed in laboratory results.
... Although GN is generally considered to occur more frequently in young people aged 10 to 40 years old, without sexual predominance [3], some recent studies have shown that it may also be seen between the ages 40 and 50 [6]. AGN are usually asymptomatic (62.9% to 93.3% of cases) [6], and often discovered incidentally as they are slow growing and usually endocrinologically inactive [7]. ...
... Although GN is generally considered to occur more frequently in young people aged 10 to 40 years old, without sexual predominance [3], some recent studies have shown that it may also be seen between the ages 40 and 50 [6]. AGN are usually asymptomatic (62.9% to 93.3% of cases) [6], and often discovered incidentally as they are slow growing and usually endocrinologically inactive [7]. However, GN can grow large enough to cause symptoms due to mass effect as was demonstrated in our patient. ...
... A PET scan is one of the most helpful modalities to differentiate malignancy and adenoma with 100% sensitivity and 98% specificity [16]. In reality, these radiological features are non-pathognomonic of adrenal GN [15], so the preoperative misdiagnosis rate of adrenal GN based on CT and MRI results was confirmed at 64.7% [6]. Thus, the preoperative differential diagnosis of GN (with ganglioneuroblastoma, neuroblastoma, pheochromocytoma, adenoma, and adrenocortical carcinoma) remains extremely challenging [5,17]. ...
Article
Adrenal ganglioneuroma (AGN) are sympathetic differentiated tumors that originate from neural crest cells. It is a rare benign tumor in children and young adults. These lesions are usually asymptomatic and tend to be hormonally silent. Their discovery is fortuitous in imaging examinations. Preoperative diagnosis remains difficult, and the gold standard treatment is adrenalectomy. There is a good prognosis after surgery without recurrence. We herein report a case of adrenal ganglioneuroma in a 40-year-old man who benefited from an abdominal CT scan in the face of a complaint of abdominal discomfort and as part of the extension assessment of his colonic adenocarcinoma. Abdominal CT scan with contrast showed a left retroperitoneal mass of triangular shape within the adrenal lodge of tissue density, containing some calcifications not enhanced after injection of contrast product, measuring 90 x 62 mm in diameter (AP x T) with a relative washout calculated at 30%. Biopsy and histological examination of the mass suggested an adrenal ganglioneuroma.
... They originate from primordial neural crest cells and develop as primary neoplasms or from differentiation of immature neurogenic tumors such as neuroblastomas 1, 2 . Ganglioneuromas are usually hormonally silent, although some have been reported to secrete vasoactive intestinal peptide (VIP), cortisol, catecholamines, and/or testosterone [3][4][5][6][7][8][9][10] . Moreover, ganglioneuromas have been occasionally found in the setting of familial syndromes, including [11][12][13][14][15][16][17][18][19] . ...
... Pre-operative diagnosis of ganglioneuromas is challenging because these tumors have no defined clinical signs and symptoms, laboratory findings, or imaging characteristics; this may lead to misdiagnoses that result in inappropriate treatment courses 6,20,21 . As benign tumors, ganglioneuromas are often discovered incidentally or during work-up for non-specific symptoms caused by mass effect 10,22,23 . ...
Article
Objective: The Transatlantic Australasian Retroperitoneal Sarcoma Working Group (TARPSWG) conducted a retrospective study on the disease course and clinical management of ganglioneuromas. Background: Ganglioneuromas are rare tumors derived from neural crest cells. Data on these tumors remain limited to case reports and single-institution case series. Methods: Patients of all ages with pathologically confirmed primary retroperitoneal, intra-abdominal, and pelvic ganglioneuromas between January 1, 2000 and January 1, 2020 were included. We examined demographic, clinicopathologic, radiologic characteristics as well as clinical management. Results: Overall, 328 patients from 29 institutions were included. The median age at diagnosis was 37 years with 59.1% of patients being female. Symptomatic presentation comprised 40.9% of cases, and tumors were often located in the extra-adrenal retroperitoneum (67.1%). At baseline, the median maximum tumor diameter was 7.2 cm. One hundred sixteen (35.4%) patients underwent active surveillance while 212 (64.6%) patients underwent resection with 74.5% of operative cases achieving an R0/R1 resection. Serial tumor evaluations showed that malignant transformation to neuroblastoma was rare (0.9%, N=3). Tumors undergoing surveillance had a median follow-up of 1.9 years, with 92.2% of ganglioneuromas stable in size. With a median follow-up of 3.0 years for resected tumors, 84.4% of patients were disease-free following resections while recurrences were observed in 4 (1.9%) patients. Conclusion: Most ganglioneuromas have indolent disease courses and rarely transform to neuroblastoma. Thus, active surveillance may be appropriate for benign and asymptomatic tumors particularly when the risks of surgery outweigh the benefits. For symptomatic or growing tumors, resection may be curative.
... Due to the unusual location of the tumour, difficult diagnosis and the slow, but steady increase in tumour's size in the MRI, it has been decided to perform a coarse-headed biopsy. Nishihira et al. suggest that the slow growth of the tumour may be result of stromal Shwann's cells' proliferation and not the hyperplasia of malignant tissue [11,13] . This could explain the, observed in subsequent MRI, slight increase in the size of the tumour, without any evidence of disease progression -as the tumour's growth is not greater than 25%. ...
... The method of choice in the treatment of ganglioneuroma is a surgery with total or subtotal resection of the tumour, having a similar prognosis to the incomplete resection with less than 2 cm residual disease [13,14] . Adjuvant chemotherapy does not affect the progression-free survival or overall survival in any way. ...
Article
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Ganglioneuroma is a rare tumour originating from neural crest cells, occurring mainly within children older than 7 years. It can be localised in pelvic; however, this localisation is extremely rare. This paper presents the case of a 39-year-old woman, at whom the pelvic localisation of the lesion and the unspecific symptoms associated with the digestive and genital tract impeded the recognition of the actual disease. The immensely slow growth of the tumour, combined with gradual fading of the symptoms, indicated its benign character. Only the CT-controlled biopsy enabled the recognition of the ganglioneuroma. Taking under consideration the histopathologic result and the cease of the symptoms, we decided to leave the patient under observation. After 6 years of observation, no progression signs have been recorded.
... A case of increased FDG uptake (SUVmax 4) occurred in a patient with a history of breast cancer, and the incidental adrenal mass originally was thought to be a metastasis. 7 Two reported cases of AGN showed mild increased FDG uptake (SUVmax 2.5). 5 In two cases from our own institution, one AGN had no increased FDG uptake and the other had very mild increased uptake (SUVmax 1.5) (Fig 4b). ...
Article
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Although adrenal medullary tumours are rare, they have important clinical implications. They form a heterogeneous group of tumours, ranging from benign, non-secretory, incidental masses to hormonally active tumours presenting acutely, or malignant tumours with disseminated disease and a poor prognosis. Increasingly, benign masses are incidentally detected due to the widespread use of imaging and routine medical check-ups. This review aims to illustrate the multimodality imaging appearances of rare adrenal medullary tumours, excluding the more common phaeochromocytomas, with clues to the diagnosis and to summarise relevant epidemiological and clinical data. Careful correlation of clinical presentation, hormone profile, and various imaging techniques narrow the differential diagnosis. Image-guided percutaneous adrenal biopsy can provide a definitive diagnosis, allowing for conservative management in selected cases. A close collaboration between the radiologist, endocrinologist, and surgeon is of the utmost importance in the management of these tumours.
... Ganglioneuromas typically arise from primordial neural crest cells present in the adrenal medulla [28][29][30]. Calcifications may be apparent on CT-scan in 30%-60% of cases. Unenhanced attenuation values are relatively high: >25 HU. ...
Article
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The finding of an adrenal mass should induce a diagnostic work-up aimed at assessing autonomous hormone production and differentiating between benign and (potentially) malignant lesions. The common differential diagnosis in adrenal incidentaloma consists of (non-)functioning adenoma, pheochromocytoma, myelolipoma, metastasis, and primary carcinoma. There remains a category of lesions that are hormonally inactive and display nonspecific imaging characteristics. We provide a succinct literature review regarding pathologies from this category. Imaging and histological characteristics are discussed, as well as clinical management. In conclusion, an adrenal mass may present a diagnostic challenge. After exclusion of most common diagnoses, it can be difficult to differentiate between possible pathologies based on preoperative diagnostic tests. Surgical resection of possibly harmful tumors is indicated, for example, lesions with malignant potential or risk of spontaneous hemorrhage. Resection of an obviously benign lesion is not necessary, unless problems due to tumor size are expected.
... Ganglioneuromas constitute 0.72 to 1.6 of all the tumors located in the retroperitoneal space 9,10 . Primary ganglioneuroma evolves as a mature tumor de novo and secondary ganglioneuroma found during or after the treatment of neuroblastoma 5,7,9,11,12 . ...
Article
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Ganglioneuromas are benign tumors that originate from primordial neural crest cells. We report a retroperitoneal ganglioneuroma arising from the left paraspinal region. A twenty-year old woman presented with left lumbar pain. Neurological workup was normal. Imaging revealed a left paraspinal retroperitoneal mass measuring 9.74 × 6.19 × 4.30 centimeters. Complete surgical removal was uneventful. During follow-up, left lumbar pain improved and imaging showed no evidence of the disease.
... Adrenal GNs occur most frequently in the fourth and fifth decades of life, whereas GNs of the retroperitoneum and posterior mediastinum are usually encountered in children and younger adults. GNs seem to develop in females and males with equal rates; yet most of our data derive from case reports or small case series [5][6][7] . Nonetheless, a familial predisposition as well as an association with Turner syndrome and multiple endocrine neoplasia Ⅱ have also been suggested [5] . ...
Article
Adrenal ganglioneuromas (GNs) constitute rare, differentiated tumors which originate from neural crest cells. GNs are usually hormonally silent and tend to be discovered incidentally on imaging tests. Adrenalectomy is the gold standard for the treatment of primary adrenal GNs. Nevertheless, preoperative differential diagnosis of GNs remains extremely challenging, and thus histopathological examination is required in order to confirm the diagnosis of GN. Overall, prognosis after surgical resection seems to be excellent, without any recurrences or need for adjuvant therapy.
... 22,23 Complete excision is often associated with an excellent outcome and no recurrence. 20,24 In our institution, there are 18 cases of adrenal incidentalomas with no autonomous hormonal secretion reported in the past 5 years. These include 4 cases of mature ganglioneuroma, 1 case of adenomatoid nodule, 3 cases of myelolipoma, 4 cases of haemangioma/lymphangioma, 5 cases of benign cyst and 1 case of metastatic adenocarcinoma. ...
Article
Introduction: The increasing use of radiological imaging studies has given rise to 'incidentalomas'. Case report: We describe two unusual and diverse incidental adrenal gland lesions, an adenomatoid nodule and a mature ganglioneuroma. Both are deemed 'indeterminate' on radiological assessment. On histology, an adenomatoid nodule is composed of variably-dilated thin-walled cysts lined by bland flattened cells and solid areas of tubules lined by eosinophilic cells with plump nuclei and prominent nucleoli. The lining cells are immunoreactive for calretinin and WT1 while negative for CK5/6, ERG and CD31. Mature ganglioneuroma features fascicles of bland spindle cells with intermixed mature ganglion cells disposed within a background myxoid stroma with no immature neuroblastic component. These spindled Schwann cells are S100 positive. Discussion: Both adenomatoid nodule and mature ganglioneuroma are rare benign adrenal tumours that need to be differentiated from other, more common adrenal lesions. The management of adrenal incidentalomas is challenging. Surgical excision is indicated if an adrenal incidentaloma is more than 4 cm in size, shows malignant features on imaging or evidence of hormone excess.
... Gan-glioneuroma is generally considered to occur more frequently in children or young adults 2,6) . However, more recent studies have shown that the mean age at diagnosis of ganglioneuroma is around 40 to 50 years old 5,8,12,15) . Regarding treatment, surgical decompression should be undertaken as soon as possible when spinal cord compression is present as present case 7) . ...
Article
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Ganglioneuroma is an uncommon benign tumor and arise from neural crest cells or sympathetic and peripheral nerves. A 13-year-old boy was referred to our institute and presented with three-day history of right upper extremity weakness associated with neck pain for nearly one month. Magnetic resonance imaging showed right side dumbbell shaped extramedullary lesion at the C2-3 level extending outside through neural foramen. Paraspinal and posterior neck mass, cafe au lait spot was identified. We performed surgical decompression of the spinal cord by resection of intradural portion mass. Histopathological diagnosis was ganglioneuroma.
... Unlike neuroblastoma, AGN is more common in adults and less common in children. Previous studies have shown that the age of onset of AGN is between 30 and 50 years, with no significant difference in incidence between men and women [13,14]. The mean age at diagnosis of the 51 patients in this study was 41.2 years, which was generally consistent with the literature. ...
Article
Objectives: To analyze the clinical features, imaging findings, and surgical results of 51 cases of adrenal ganglioneuroma (AGN) in our center to provide a clinical reference for its diagnosis and treatment. Methods: We retrospectively reviewed the clinical data of 51 AGN patients admitted to our hospital. We summarized the clinical and imaging characteristics and analyzed the effects of different surgical methods and tumor size on the perioperative outcome. Results: The mean tumor size was 6.1±2.4 cm. The mean computerized tomography (CT) value of tumors on the plain scan images was 29.9±4.3 Hounsfield units (HU), and 60.8% (20/45) of tumors showed delayed progressive enhancement on enhanced CT images. Pathologically, immunohistochemistry revealed a positive rate of 91.7% for S-100 (11/12) and 77.8% (7/9) for Vimentin. Of the 51 patients, 32 underwent laparoscopic adrenalectomy, and 19 underwent open adrenalectomy. The median postoperative follow-up time was 51.1 (2-125) months, and no tumor recurrence or metastasis occurred during the follow-up. The estimated blood loss (EBL) was reduced in the laparoscopic group compared to the open group (P=0.027). Larger tumors prolonged the operation time and postoperative hospital stay (both P<0.05). Conclusions: This study reports the largest series of AGN patients to date. CT plays a guiding role in the preoperative diagnosis of AGN, but the pathologic results are the most reliable. Laparoscopic adrenalectomy may be safer than open surgery. Larger tumor volume is a risk factor for a longer operative time and postoperative hospital stay.
... Also, ganglioneuromas often have progressive enhancement pattern on CT that can help distinguishing ganglioneuromas from other adrenal tumors such as ACC and adenomas (Fig. 16) [101]. Because the mean size of adrenal ganglioneuromas is 6.8 cm and they commonly demonstrate heterogeneity and calcification, ganglioneuromas are usually managed surgically [102]. ...
Article
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Adrenocortical carcinoma (ACC) is a rare tumor with a poor prognosis. Most tumors are either metastatic or locally invasive at the time of diagnosis. Differentiation between ACC and other adrenal masses depends on clinical, biochemical, and imaging factors. This review will discuss the genetics, pathological, and imaging feature of ACC.
... 8 Complete surgical excision reaching negative tissue margin results in excellent outcome and is the treatment of choice in malignant cases. 9 The surgical resection of these tumors can be undertaken via laparoscopic or open technique. Laparoscopic surgery is increasingly becoming common for retroperitoneal pathologies due to increasing expertise and advanced energy sources and instruments. ...
Article
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Ganglioneuromas arise from neural crest sympathogonia and are rare benign neurogenic tumors. The most common affected sites are posterior mediastinum and the retroperitoneum. They rarely affect adrenal glands. Ganglioneuromas often present as solitary, painless and slow growing mass and are benign in nature. These tumors are closely related to major vessels. Hence, surgical management of retroperitoneal pathologies may require multiorgan resection in order to achieve complete surgical resection while preservation of surrounding organs especially in case of benign tumors. We present a case report of a 21 year old male with a 12×10×10 cm size right sided retroperitoneal ganglioneuroma which on computed tomography (CT) mimicked adrenal tumor crossing the midline and abutting the aorta and splaying the inferior venal cava and renal vein. Present paper is an attempt to review the various surgical options available while dealing with these benign retroperitoneal tumors which are related closely to retroperitoneal organs and major vessels.
... The biggest resected GN assessable through literature research up to now showed a maximum diameter of approximately 23 cm [6] in a 42-year-old patient located solely thoracically [6]. Located most commonly in the posterior mediastinum (41.5%) or retroperitoneally (37.5%), ganglioneuromas can be found in the adrenal glands (21%), in the neck (8%), retropharyngeally, or more rarely in the sella turcica [7][8][9][10]. Computed tomography (CT) or magnetic resonance imaging (MRI) represents the gold standard for diagnosis and estimation of tumor extent. ...
Article
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Background: Ganglioneuromas (GNs) are extremely rare, slowly growing, benign tumors that can arise from Schwann cells, ganglion cells, and neuronal or fibrous tissues. Due to their origin from the sympathetic neural crest, they show neuroendocrine potential; however, most are reported to be hormonally inactive. Nevertheless, complete surgical removal is recommended for symptom control or for the prevention of potential malignant degeneration. Case report: A 30-year-old female was referred to our oncologic center due to a giant retroperitoneal and mediastinal mass detected in computed tomography (CT) scans. The initial symptoms were transient nausea, diarrhea, and crampy abdominal pain. There was a positive family history including 5 first- and second-degree relatives. Presurgical biopsy revealed a benign ganglioneuroma. Total resection (TR) of a 35 × 25 × 25 cm, 2550-g tumor was obtained successfully via laparotomy combined with thoracotomy and partial incision of the diaphragm. Histopathological analysis confirmed the diagnosis. Surgically challenging aspects were the bilateral tumor invasion from the retroperitoneum into the mediastinum through the aortic hiatus with the need of a bilateral 2-cavity procedure, as well as the tumor-related displacement of the abdominal aorta, the mesenteric vessels, and the inferior vena cava. Due to their anatomic course through the tumor mass, the lumbar aortic vessels needed to be partially resected. Postoperative functioning was excellent without any sign of neurologic deficit. Conclusion: Here, we present the largest case of a TR of a GN with retroperitoneal and mediastinal expansion. On review of the literature, this is the largest reported GN resected and was performed safely. Additionally, we present the first systematic literature review for large GN (> 10 cm) as well as for resected tumors growing from the abdominal cavity into the thoracic cavity.
... The majority of AGN are non-secretory; there are fewer Case Report Dopamine secreting adrenal tumor-ganglioneuroma rather than pheochromocytoma: case report than 10 documented cases of pure hormone-secreting AGN in adults in English literature (8)(9)(10)(11)(12)(13)(14)(15)) ( Table 1). ...
Article
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Ganglioneuromas are rare, benign, well-differentiated neural crest tumors arising in the paravertebral sympathetic chain, and are classically non-secretory and clinically asymptomatic. As the diagnosis of ganglioneuroma is based on histopathology, the clinical presentation prior to surgical excision often mirrors that of pheochromocytoma or adrenal cortical adenoma. We describe a case of an incidentally found right sided calcified adrenal mass with evidence of marked dopamine excess, suspicious for pheochromocytoma in a 70-year-old female. The patient endorsed a 6-month history of intermittent right flank pain and a 2-year history of weight loss and fatigue. She reported mild symptoms of hypomania but denied other symptoms of dopamine excess including agitation, anxiety, nausea, and vomiting. Exam revealed isolated mild hypertension. The imaging features of this mass were concerning for malignancy including the presence of macrocalcification and irregular borders. After preoperative alpha blockade, the patient underwent open right adrenalectomy and the final pathology was consistent with ganglioneuroma rather than pheochromocytoma. Following resection, the dopamine level normalized, confirming the resected right adrenal ganglioneuroma as the source of dopamine excess. This case represents a rare presentation of dopamine-secreting adrenal ganglioneuroma. This illustrates that although rare, ganglioneuroma should be included on the differential diagnosis for functional adrenal tumors.
... Кальцинаты, по данным литературы, представлены в 0-29% случаев (рис. 11) [27]. ...
Article
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The adrenal incidentaloma is a lesion of a different etiology and found incidentally in patients who underwent a diagnostic study not about the disease of this organ. Lesions can be both hormonally inactive and hormonally active, can arise from different zones of the adrenal gland or have non-specific organ affiliation, can be benign or malignant. Computed tomography characterization of these lesions, especially the differential diagnosis of benign and malignant, is extremely important for the correct diagnosis in order to provide adequate management of the patient. The article presents the key computed tomography criteria that allow radiologist to characterize the lesion most accurately and consider appropriate diagnosis.
... Since GNs are mostly hormone-silent and asymptomatic and retroperitoneum space is deep and extensive, they can be huge when detected. These findings were consistent with previous studies [1,4,20]. The gender incidence of GN varies in literature [7,8,14,21,22]. ...
Article
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Background The preoperative evaluation is crucial for diagnosis and surgical plan of retroperitoneum ganglioneuroma (GN). In this study, we reviewed a relatively large series of histopathological proved retroperitoneum GN cases, summarized the imaging features and further depicted risk factors of increased surgical blood loss. Methods A total of 35 (18 male, 17 female) patients were retrospectively enrolled from January 2012 to June 2019 at our institution. Among them, 24 patients had undergone CT scans and 19 patients had undergone MR examination before treatment. The clinical and radiological features were analyzed and the relationships between image features and surgical blood loss were evaluated. Results The media age of the involved 35 patients was 40 years (range, 14–66 years). The histological tumor size was 10.12 ± 4.56 cm for average. Retroperitoneum GN was relatively low density on unenhanced CT images and showed delayed progressive enhancement on enhanced CT and MR images. The whorled sign could be seen in 14 patients. The vessel encasement sign could be found in 17 patients. Univariate analysis revealed maximal tumor size measured on axial image, maximal tumor size measured on coronal image, encasing one or both renal pedicles, encasing the aorta and/or vena cava and whorled sign on MRI showed significant difference between the blood loss ≥ 400 ml and blood loss < 400 ml group. Logistic regression further detected that maximal tumor size measured on axial images (OR: 1.12; 95% CI: 1.02–1.24; P = 0.023) and encasing one or both renal pedicles (OR: 22.39; 95% CI: 1.35–372.99; P = 0.030) were independently correlated with surgical blood loss. Conclusions Preoperative CT and MR imaging analysis was valuable for both diagnosis and surgical risk prediction of retroperitoneum GN.
... 5 7 Treatment may be by open or laparoscopic adrenalectomy. 4 Patients with PCOD present with features of androgen excess produced by ovary and sometimes by adrenal androgens due to an adenoma. So in a patient with PCOD presenting with a simultaneous adrenal mass, GN should also be a differential diagnosis. ...
Article
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Adrenal ganglioneuromas are rare, benign incidentalomas of a neural crest origin. A majority of these tumours are clinically silent and discovered on imaging for unrelated reasons. Polycystic ovarian disease (PCOD) is an endocrine disorder characterised by bilateral polycystic ovaries, anovulation leading to infertility, irregular menstrual cycles and features of androgen hormone excess. Herein we report a rare case of adrenal ganglioneuroma in a 14-year-old girl with PCOD. She was referred to us by the gynaecologist after incidental detection of adrenal mass on ultrasonography. Except for raised 24 h urinary metanephrines, rest of the hormones measured were in normal range. Transperitoneal adrenalectomy was performed and histopathology was suggestive of ganglioneuroma. Postoperative recovery was excellent and she is doing well. To our knowledge it is the first such type of case to be reported.
... The described calci cation discovery rate ranged from 30 to 60% in ganglioneuromas, whereas only small cases have reported the presence of calci cations in adenomas [29][30][31]. In our study, the detection rate of calci cation was 23.8% (10 of 42 ) in ganglioneuromas, compared with 6.0% (13 of 216) for lipidpoor adenomas (P < 0.05). ...
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Background: The utility of dual-phase enhanced CT scan in distinguishing ganglioneuromas from lipid-poor adenomas has not been reported. We aimed to prospectively compare CT findings helpful in distinguishing adrenal ganglioneuromas from adrenal lipid-poor adenomas. Methods: We estimated the CT findings of 258 adrenal masses (42 ganglioneuromas, 216 lipid-poor adenomas) in 258 patients from July 2008 to July 2020 with ganglioneuromas and July 2016 to July 2020 with lipid-poor adenomas. The CT features between ganglioneuromas and lipid-poor adenomas were compared. Results: Significant differences were detected in CT value of unenhanced (CTU), CT value of arterial phase (CTA), CT value of venous phase (CTV), degree of enhancement in arterial phase (DEAP), degree of enhancement in portal venous phase (DEPP), age, tumor size [long diameter (LD), short diameter (SD), mean diameter (MD)], shape, calcification between the ganglioneuroma and lipid-poor adenoma groups (P < 0.05). The results of receiver operating characteristics (ROC) analyses showed that areas under ROC curves (AUC) of CTU, CTA and CTV were 0.713, 0.878, and 0.914, respectively. When the cut-off values were set at 22.5 HU, 51.5 HU, and 53.5 HU for CTU, CTA, and CTV, respectively the three parameters had a sensitivity of 46.8%, 67.6%, and 88.0% and a specificity of 100%, 100%, and 88.1% in distinguishing between ganglioneuromas and lipid-poor adenomas. Conclusion: Dual-phase enhanced abdominal CT can exhibit some of the primary imaging characteristics of ganglioneuromas and lipid-poor adenomas used to distinguish between these two entities.
... Ganglioneuromas are benign and well-differentiated tumors of the sympathetic nervous system. They arise from the great sympathetic chains extending from the base of the skull to the neck, mediastinum, retroperitoneum, and adrenal glands [1] [2]. Ganglioneuromas of the adrenal gland are extremely rare. ...
... The tumor has no hormonal hypersecretion. CT scan imaging characteristics of AGNs usually help to distinguish them from other adrenal neoplasms which include: (a) low attenuated (<40 Houndfield units) homogeneous mass in the precontrast phase (b) progressive enhancement on postcontrast phase and delayed images (c) presence of punctuate or discrete calcification in about 50 percent of cases [2][3][4]. Adrenal adenomas usually have similar morphology to AGN, however they are often smaller in size with characteristic washout of more than 50% contrast on delayed images; whereas adrenocortical carcinomas are typically large (>5 cm), heterogenous on precontrast images with inhomogenous contrast enhancement. ...
Article
Evaluation of the adrenal glands is a frequent indication for cross-sectional imaging, in particular in oncology patients. The following chapter reviews adrenal gross and microscopic anatomy, imaging protocols, and an algorithmic approach to evaluating adrenal masses.
Article
Purpose: Ganglioneuromas (GNs) are benign neoplasms of combined neural crest, schwannian,and connective tissue origin, occurring rarely in the adrenal glands. The present study is to share our experience regarding diagnostic and therapeutic management of these tumors. Materials and methods: Adrenal GNs of 15 patients were found incidentally with ultrasonography and were evaluated subsequently with computed tomography (CT) scan. Clinical data as well as follow-up data were collected retrospectively. All the patients received operative resection. Results: The mean age of the patients was 38.4 years (range, 25-52 years; male to female ratio, 2:1). Of study subjects 11 patients had unilateral GN on the right side, and the remaining 4 on the left side. All but 1 patient were asymptomatic. No hormonal secretion was apparent. Mean size of the tumors in CT scan was 6.27 cm (range, 2.5-14 cm), while 10 were larger than 5 cm. Eight patients underwent open adrenalectomy and the remaining 7 underwent laparoscopic anterior adrenalectomy. Histologically, all 15 neoplasms were completely differentiated,mature GN. We had no mortality or significant morbidity. Mean duration of hospitalization was 5.5 days (range, 3-7 days). There was no recurrence, during a mean followup of 5.4 years (range, 1-10 years). Conclusion: Pre-operative diagnosis of adrenal GNs remains difficult merely according to physical examination. Therefore, we recommend complete operative resection once malignancy cannot be excluded by pre-operative analyses. Laparoscopic adrenalectomy is a reasonable option, at least for tumors ≤ 5 cm.
Article
A 32-year-old female with a history of hypertension and paroxysmal supraventricular tachycardia (PSVT) presented with a 63×39 mm, well-circumscribed retroperitoneal mass found by ultrasonography and abdominal computed tomography (CT). CT revealed a round, homogeneous tumor with calcification at the left renal hilum, and compressing the left adrenal and renal veins. Endocrinological examinations were within the normal range. Transabdominal laparoscopic excision of the retroperitoneal mass was planned because tumor malignancy could not be excluded by preoperative analyses. Histopathological examination proved that the tumor was a ganglioneuroma arising from the extra-adrenal retroperitoneum.
Article
Objective: The purpose of this review is to describe FDG uptake characteristics of adrenal lesions, which can show increased FDG uptake on PET/CT. Conclusion: Both benign and malignant adrenal lesions can show increased FDG uptake. Knowledge of the uptake characteristics of these lesions is helpful for increasing diagnostic accuracy and expanding the differential diagnosis for adrenal lesions.
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PET/CT with (18)F-fluorodeoxyglucose (FDG) or using different radiocompounds has proven accuracy for detection of adrenal metastases in patients undergoing cancer staging. It can assist the diagnostic work-up in oncology patients by identifying distant metastases to the adrenal(s) and defining oligometastatic disease that may benefit from targeted intervention. In patients with incidentally discovered adrenal nodules, so-called adrenal "incidentaloma" FDG PET/CT is emerging as a useful test to distinguish benign from malignant etiology. Current published evidence suggests a role for FDG PET/CT in assessing the malignant potential of an adrenal lesion that has been 'indeterminately' categorized with unenhanced CT, adrenal protocol contrast-enhanced CT, or chemical-shift MRI. FDG PET/CT could be used to stratify patients with higher risk of malignancy for surgical intervention, while recommending surveillance for adrenal masses with low malignant potential. There are caveats for interpretation of the metabolic activity of an adrenal nodule on PET/CT that may lead to false-positive and false-negative interpretation. Adrenal lesions represent a wide spectrum of etiologies, and the typical appearances on PET/CT are still being described, therefore our goal was to summarize the current diagnostic strategies for evaluation of adrenal lesions and present metabolic and anatomic appearances of common and uncommon adrenal lesions. In spite of the emerging role of PET/CT to differentiate benign from malignant adrenal mass, especially in difficult cases, it should be emphasized that PET/CT is not needed for most patients and that many diagnostic problems can be resolved by CT and/or MR imaging.
Article
I-MIBG accumulation in focal nodular hyperplasia was demonstrated. A 16-year-old girl who received radiotherapy for neuroblastoma at the age of 5 presented for abdominal pain. Three MIBG-avid tumors within the liver were proven to be focal nodular hyperplasias through biopsy. Previous neuroblastoma was transformed to non-MIBG-avid ganglioneuroma. MIBG accumulation does not necessarily indicate the presence of neuroendocrine tumor.
Article
Objective: To analyze CT appearances of retroperitoneal neurogenic tumors. Methods: Totally 34 patients with neurogenic tumors confirmed by surgical pathology underwent plain and contrast enhance CT scanning before operation. CT findings were analyzed. Results: Among 34 cases, tumors located in adrenal gland area in 25 patients, while out of the adrenal gland area in 9 patients. Three malignant tumors were found in 14 lobulated tumors, and 4 malignant tumors were found in 20 round tumors. Calcifications were detected in 7 cases, including 4 neuroblastomas and 3 ganglioneuromas. Irregular calcifications were found in neuroblastomas, and tiny calcifications were found in ganglioneuromas. Conclusion: CT findings of retroperitoneal neurogenic tumors show some definite characteristics, which contribute to correct diagnosis combined with the age and clinical manifestations. Copyright©2013 by the Press of Chinese Journal of Medical Imaging Technology.
Article
Objective: To explore CT and MRI features of ganglioneuromas in children. Methods: Clinical and imaging data of 23 children with ganglioneuroma confirmed by histopathology after surgical operation were retrospectively reviewed. Plain CT scan was performed in 23 children, while early enhanced CT scanning was performed in 21 and delayed phase in 9. Seven among these 23 children underwent plain MR imaging, while 4 of them underwent enhanced MR scanning. Results: Among 23 patients, 17 ganglioneuromas were detected in retroperitoneum, 5 in mediastinum, and 1 in neck. All tumors presented oval or crescent-shaped with well defined border on both CT and MR images. The tumors predominantly showed low attenuation on plain CT images, with the CT value ranged from 22 to 38 HU (median 28 HU), and hypointensity on T1WI and inhomogeneous hyperintensity on T2WI. Post-contrast enhancement on both CT and MR images was lacking or slight in early phase, but marked in late phase. Conclusion: Low attenuation on plain CT images, inhomogeneous hyperintensity on MR T2WI images and marked enhancement in late phase are imaging features of ganglioneuromas in children.
Article
The aim of this study is to review and evaluate the clinical and pathological features of nonfunctioning adrenal tumors treated by surgical excision. Between January 1997 and December 2009, adrenalectomy was performed for 113 patients (115 adrenal glands) at Nagasaki University Hospital. Of these, 13 patients (14 adrenal glands) were characterized as hormonally inactive. There were seven male patients and six female patients with a mean age of 57 years. The mean size of the tumors was 5.2 cm (range, 2-9 cm). Among them, five lesions were diagnosed as adrenal malignancies histopathologically, including four metastatic adrenal tumors (mean size, 3.5 cm; range, 2-4.8 cm). The other 9 cases included four myelolipomas, three non-functioning adrenocortical adenomas and two ganglioneuromas. Nine patients, including two with three adrenal metastases, underwent laparoscopic adrenalectomy (mean tumor size, 5 cm; range, 2-9 cm). Generally, the indication for surgery in patients with a nonfunctioning adrenal tumor is based upon the tumor size and the image findings. Careful decision making is required for surgical intervention, because some cancer patients may have small adrenal metastatic lesions. In addition, laparoscopic adrenalectomy is a feasible procedure for the resection of a large adrenal tumor of more than 6 cm, based on careful patient selection.
Article
Aim: To analyse the computed tomography (CT) imaging features of patients with adrenal schwannoma. Materials and methods: Eight cases of adrenal schwannoma confirmed by histopathology were included in this study. All eight patients had undergone multiphase CT examinations. The features of the adrenal schwannoma in the CT images were analysed retrospectively in detail, including size, shape, margin, radiodensity, calcification, and enhancement pattern. Results: There were six male and two female patients, with a median age of 44.5 years (range, 25-52 years). Two patients complained of right flank pain, and two with left upper abdominal discomfort, while the remaining patients were diagnosed by routine ultrasound examinations. On unenhanced CT images, all cases of adrenal schwannoma were well circumscribed, rounded or oval, heterogeneous masses with cystic components, with two cases exhibiting calcification, and three cases with septa. On enhanced CT images, all cases displayed mild heterogeneous enhancement of the tumour during the arterial phase, and progressive enhancement during the portal venous phase and equilibrium phase. Conclusion: Adrenal schwannoma commonly presents as a well-defined unilateral mass with cystic degeneration, septa, and a characteristic progressive contrast-enhancement pattern on multiphase enhanced scans.
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While accidentally detecting an adrenal gland lesion (incidentaloma) during a routine computed tomography (CT) scan, the radiologist should correctly interpret revealed changes. The most common lesion is adenoma with high lipid content, but a lipid poor adenoma, pheochromocytoma, adrenocortical cancer, metastasis and other less common adrenal diseases are also worth of attention and require detailed knowledge of their CT semiotics. The article presents criteria of differential diagnosis of the adrenal incidentalomas on the basis of which an algorithm of differential diagnosis was proposed for the most common adrenal lesions.
Article
Background Adrenal ganglioneuroma (AGN) is a rare tumor that originates from the gangliar cells of the sympathetic nervous system. It represents less than 5% of all adrenal masses. AGN occurs as a large mass, with benign behavior and no relevant symptoms and hormonal secretion, but it is often misdiagnosed because the preoperative radiological diagnosis is generally challenging. The aim of the present paper is to report the experience of a tertiary referral academic center regarding the management of AGN and review the relevant literature. Methods The demographic, diagnostic, surgical, and pathological findings of 666 consecutive patients who were adrenalectomized at the Padua Endocrine Surgery Unit between 1990 and 2015 were retrospectively reviewed in order to focus on AGN. Results The pathology confirmed AGN in 10 patients (1.5% of cases; median age 35 years, range 19–73). The diagnosis was incidental and never available before surgery. Eight patients were asymptomatic, two presented lower back and abdominal pain. The mass was non-secreting in all cases. Preoperative imaging techniques were inconclusive about the nature of the mass in all cases. The median size of the tumor was 55 mm (range 30–80). The laparoscopic approach was performed in 60% of cases. No recurrences occurred after surgery at a median follow-up of 10 years (range 1.7–18.2). Conclusion The diagnosis of AGN may be challenging and most often occurs after surgery, since it is frequently confirmed by histological examination. Radical excision may be achieved by laparoscopic adrenalectomy, with a subsequent definitive cure and excellent prognosis at long term follow up.
Article
Background: Adrenal ganglioneuromas (AGNs) are extremely rare benign neoplasms. This study is to share our experience regarding diagnostic and operative management of these tumors. Methods: Clinical details as well as follow-up data were retrospectively analyzed in 42 primary AGN patients who received operative resection at a tertiary referral hospital in China between May 2005 and July 2016. Results: The mean age of the patients at diagnosis was 35.3 years (range 13-59 years). Among the patients, 28 were asymptomatic. The mean preoperative size was 5.3 ± 2.2 cm (range 2.1-14.0 cm), and 17 cases were larger than 5 cm. All patients underwent operative resection as open adrenalectomy in 20 patients and laparoscopy in 22 patients. The mean tumor size on pathologic examination was 6.0 ± 2.6 cm (range 2.2-17.0 cm). Laparoscopic operation showed its advantages over open surgery on the postoperative duration (5.0 vs. 7.3 days, p < 0.001) and estimated blood loss (69.5 vs. 157.5 ml, p = 0.047). No patient had recurrence or metastasis during a median follow-up of 70.5 months (range 3.7-164.3 months). Conclusions: We describe the largest AGN series reported to date. The preoperative diagnosis of AGN remains difficult despite of the progression of imaging examination. After complete resection, the prognosis of AGN patients is excellent. Laparoscopic approach has its advantages in the resection of AGNs.
Article
Ganglioneuroma is a benign well differentiated neural tumor, and arises from ganglion cells of the sympathetic nerve. It lacks clinical symptoms and has often enlarged when it is detected. The tumor has increasingly been found as an adrenal incidentaloma. We, herein, report a case of ganglioneuroma of the right adrenal gland. A 47-year-old woman was referred to our hospital for an incidentally found right adrenal tumor of 45 mm in maximum diameter. No abnormalities in the endocrine function or images suggestive of malignancy were found. Right nonfunctioning adrenal tumor was considered. Laparoscopic right adrenalectomy conducted to make the definite diagnosis revealed that the tumor was a ganglioneuroma of the adrenal gland consisted of spindle shaped tumor cells and nests of ganglion cells without atypia. Diagnostic and therapeutic laparoscopic adrenalectomy could feasibly be considered for relatively large (>4 cm in maximum diameter) adrenal tumors with non-typical imaging features.
Article
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Background Ganglioneuromas are very rare tumors of the sympathetic nervous system. Clinical and pathological knowledge is currently based on largely incomparable registries and case series that focus on pediatric or adrenal cases. To comprehensively characterize the full clinical spectrum across ages and locations, a meta‐analysis was performed where amenable and complemented by systematic literature review of individual patient data (IPD). Design Articles containing “ganglioneuroma” in English on humans, published from 1/1/1995‐6/27/2018 were identified from Pubmed. Aggregate data from 10 eligible patient series on 19 variables were considerably inhomogeneous, restricting meta‐analysis to age and gender distribution. To determine basic disease characteristics across ages and locations IPD were retrieved from case reports and small case series (PROSPERO CRD42018010247). Results IPD representing 364 cases revealed that 65.7% (60.6‐70.4%) were diagnosed in adults, more frequently in females (62%, 56.9‐66.9%). 24.5% (20.3‐39.1%) were discovered incidentally. Most often ganglioneuromas developed in abdomen/pelvis (66.2, 32.1% adrenal). With age the proportion of ganglioneuroma localizations with high post‐surgical complication rate (35.6% head/neck and 16.3% thorax) decreased. Contrarily, the diagnosis of adrenal ganglioneuromas (<1% post‐surgical complications) increased with age. Hormone‐production, hypertension, or coincidence with another non‐neuroblastic neural‐crest‐derived tumor component was more common for adrenal location. Recurrence and metastatic spread have not been reported for ganglioneuromas without secondary tumor component. Conclusions This work summarizes characteristics of the currently largest number of international GN patients across all ages. The data confirm a benign nature of GN, independent of age. Age related differences in predominant tumor location, associated post‐surgical complications, and hormone production suggest case‐centered management strategies.
Article
Background: Ganglioneuroma is a rare tumor derived from the neural crest that can occur in any sympathetic tissue. It corresponds to 0.3% to 2% of incidental adrenal tumors and <250 have been reported in the literature so far. Case Presentation: We present a case of a 30-year-old Caucasian woman presented with a large bilobed adrenal tumor found on a CT scan during the investigation of acute abdominal pain. The image also showed a rare anatomic variation of a left-sided inferior vena cava. Biochemical work-up for adrenal incidentaloma showed normal markers. Since we could not rule out malignancy, the patient was subjected to laparoscopic adrenalectomy and the pathology report showed an adrenal ganglioneuroma, a rare nonfunctioning tumor of the adrenal. Conclusion: Ganglioneuroma can present as a large bilobed adrenal tumor. The laparoscopic approach is feasible and safe. Preoperative planning is needed and vascular variations can be challenging during the procedure.
Article
Purpose: We reviewed the experience with adrenal ganglioneuroma (AGN) pathologically confirmed following adrenalectomy in medium- to high-volume medical centers. Methods: The medical records of all adrenalectomy cases in 4 medical centers between 2006 and 2020 were retrospectively reviewed for demographics, clinical, radiological and laboratory findings, surgical treatment, pathology results, and outcomes. Results: Twenty-five out of 875 adrenalectomy cases (2.9%) were pathologically confirmed as AGN. Those patients' average age was 40.5 years (range, 4-76 years), 13 (52.0%) were males, and 18 lesions (72.0%) were right-sided. One patient had a family history of neurofibromatosis, and another had a succinate dehydrogenase gene mutation. Abdominal/back pain attributed to mass effect was the most common symptom. All 25 patients underwent abdominal computerized tomography scanning in which the average maximal tumor diameter was 6.61 cm. The mean pre- and postcontrast Hounsfield units (HU) values were 35.2 and 59, respectively; and the mean late-phase HU value was 71.1. Twenty-two patients (88.0%) underwent minimally invasive surgery. The average tumor diameter recorded in the final pathology report was 7 cm. Isolated AGN was diagnosed in 21 cases (84.0%), and the additional components reported for the remaining 4 cases included pheochromocytoma (2), ganglioneuroblastoma (1), and neurofibroma (1). The average follow-up length was 16.8 months (range, 1-136 months), during which there was no recurrence or death. Conclusion: AGN is a rare, slow-growing, large benign tumor with radiological characteristics similar to those seen in malignant tumor. Final diagnosis is established by pathology after surgical resection, preferably minimally invasive, with an overall excellent prognosis.
Article
Primary adrenal tuberculosis (TB) is a rare type of extrapulmonary tuberculosis (EPTB). A pathological biopsy is usually required to make a definite diagnosis due to nonspecific symptoms. Antituberculous chemotherapy is the main treatment regimen, and cortisol replacement therapy should be added when adrenal insufficiency is involved. Here, we present a 59-year-old man who had recurrence of oral and genital aphthosis for 3 years and was diagnosed with Behcet's disease (BD), which was cured by thalidomide. After 10 days of admission, the patient had sudden abdominal pain in the right upper quadrant with high fever and was diagnosed with acute cholecystitis attack, which was treated by percutaneous transhepatic gallbladder drainage (PTGBD). Further contrast-enhanced CT showed a right adrenal mass with a diameter of 2.0 cm, and PET-CT indicated intense 18F-fluorodeoxyglucose (18F-FDG) uptake in the right adrenal mass with a maximum standardized uptake value (SUVmax) of 15.2. As a metastatic adrenal mass was suspected, the patient underwent retroperitoneal laparoscopic adrenalectomy. Histopathological and immunohistochemical analysis revealed primary adrenal TB. After routine anti-tuberculosis treatment with isoniazid, rifampin, pyrazinamide and ethambutol for six months, the patient was cured and discharged. In summary, primary unilateral adrenal TB without adrenal insufficiency is difficult to diagnose only on the basis of clinical manifestations and examinations. Further studies are needed to develop an easier and more accurate diagnostic examination.
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Neuroblastoma is a heterogeneous disease, with manifestations ranging from spontaneous regression to lethal spread. Sometimes the tumor spontaneously differentiates toward a benign ganglioneuroma (maturing neuroblastoma). The prognosis is frequently related to ploidy, deletions in the short arm of chromosome 1, and amplifications of the N-myc oncogene. Maturing neuroblastomas consist of both neuronal cells and Schwann cells. We investigated the genetic composition of both cell types in maturing neuroblastomas, to determine the relation between genetic abnormalities and maturation. We studied 20 maturing and mature neuroblastomas by in situ hybridization to count the chromosomes and evaluate possible deletions in the short arm of chromosome 1 in neuronal and Schwann cells. The DNA content of the cells was measured by flow cytometry. Neuroblastic and ganglionic cells showed aberrations in the number of chromosomes. In situ hybridization and flow cytometry demonstrated near-trip-loidy in 18 of 19 tumors and pentaploidy in the remaining tumor. The Schwann cells in all 20 neuroblastomas contained normal numbers of chromosomes. In 18 tumors studied, there were no chromosome 1 deletions in either type of cell. The Schwann cells in maturing neuroblastomas differ genetically from the neuronal cells. The normal number of chromosomes in Schwann cells and the abnormal number in neuroblastic ganglionic cells suggests that Schwann cells are a reactive population of normal cells that invade the neuroblastoma. Near-trip-loidy of neuroblastoma cells and intact chromosome 1 are presumably genetic prerequisites for spontaneous organoid maturation, because we found no diploidy or chromosome 1 depletions in the neuronal cells of spontaneously maturing neuroblastomas.
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Ganglioneuroma is generally considered to be a benign tumor and potentially surgically curable. The authors present a case of a 21-year-old woman who underwent resection of a retroperitoneal ganglioneuroma and developed spinal neuroblastoma 11 years later. She has survived 10 more years with only recent development of metastases. To the authors' knowledge, this is the first report of malignant transformation of a ganglioneuroma into a neuroblastoma. Also, such long-term survival in an adult with spinal neuroblastoma has not been reported previously. This case raises the possibility of a dedifferentiating potential for ganglion cells in a ganglioneuroma or the presence of a long-term, quiescent form of neuroblastoma.
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There is a broad spectrum of neurogenic tumors that involve the abdomen. These tumors can be classified as those of (a) ganglion cell origin (ganglioneuromas, ganglioneuroblastomas, neuroblastomas), (b) paraganglionic system origin (pheochromocytomas, paragangliomas), and (c) nerve sheath origin (neurilemmomas, neurofibromas, neurofibromatosis, malignant nerve sheath tumors). Abdominal neurogenic tumors are most commonly located in the retroperitoneum, especially in the paraspinal areas and adrenal glands. All of these tumors except neuroblastomas and ganglioneuroblastomas are seen in adult patients. Abdominal neurogenic tumor commonly manifests radiologically as a well-defined, smooth or lobulated mass. Calcification may be seen in all types of neurogenic tumors. The diagnosis of abdominal neurogenic tumor is suggested by the imaging appearance of the lesion, including its location, shape, and internal architecture. Benign and malignant neurogenic tumors are difficult to differentiate unless distant metastatic foci are seen. For malignant tumors, imaging modalities other than computed tomography (CT) and magnetic resonance (MR) imaging may be necessary for staging. However, because most neurogenic tumors in adults are benign, CT and MR imaging can be used to develop a differential diagnosis and help determine the immediate local extent of tumor.
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Ganglioneuromas (GNs) are neural crest cell-derived tumors and rarely occur in the adrenal gland. There are presently no markers that can reliably distinguish benign and malignant neuroendocrine tumors. Here we describe a 63-year-old woman who developed sudden chest pain and hypertension combined with increased stool frequency. An incidental adrenal mass 5 cm in size with a bright signal on T2-weighted magnetic resonance imaging was discovered. Biochemical evaluation and (131)I-metaiodobenzylguanidine (MIBG) scintigraphy were negative. Histopathological examination revealed a mature adrenal GN. Neuroblastoma, the immature form of a GN, is known for deletions on chromosomal locus 1p36, and adrenal tumors frequently show allele loss on 17p. To further elucidate the histo- and pathogenesis of adrenal GN, we performed loss of heterozygosity studies on chromosomal loci 1p34-36 and 17p13 (the p53 gene locus) after careful microdissection of tumor and normal tissue. We did not detect allelic losses at these loci with the informative polymorphic markers used, suggesting that these loci are not involved in tumorigenesis. In addition, immunohistochemical investigation of the GN was positive for vasoactive intestinal peptide, a hormone commonly expressed in ganglion cells. We suggest that in our patient with an adrenal GN, the combination of biochemical, scintigraphic, molecular, immunohistochemical, and histopathological findings are all consistent with the benign morphology of this tumor.
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The size of adrenal tumors has been shown to be a good predictor of malignancy. There is still some controversy about the concordance between radiologic and real pathologic measurements. The aim of this study is to determine the correlation between direct and corrected radiologic computed tomography scan dimensions and the measurements of the resected specimen. A total of 41 adrenal tumors were included. Direct and corrected measurements of the largest diameter were contrasted with the pathologic dimensions. The Linos formula was used for the corrected measurements. Proper statistics were used considering a two-tailed significance level of 0.05. The intraclass correlations using direct and corrected measurements were 0.89 [95% confidence interval (95%CI) 0.81-0.94, p = 0.00001) and 0.90 (95%CI 0.82-0.95, p = 0.00001), respectively. The bivariate analysis using Pearson's correlation between two-dimensional group variables showed r = 0.82 (p < 0.0001) when direct and pathologic measurements were compared and r = 0.83 (p < 0.0001) when the corrected values were compared with the real dimensions. In this study, we demonstrate good correlation between radiologic and pathologic measurements of adrenal tumors. The Linos formula turned out to be significantly more accurate than direct radiologic measurements when means of the groups were compared, whereas when individual correlations were determined the two were similar. The Linos formula and radiologic measurements can be used to determine the proper management of adrenal incidentalomas in individual patients.
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The purpose of this study was to assess the role of PET with (18)F-FDG in differentiating benign from metastatic adrenal masses detected on CT or MRI scans of patients with lung cancer. This retrospective study analyzed (18)F-FDG PET scans of patients with lung cancer who were found to have an adrenal mass on CT or MRI scans. One hundred thirteen adrenal masses (75 unilateral and 19 bilateral; size range, 0.8-4.7 cm) were evaluated in 94 patients. PET findings were interpreted as positive if the (18)F-FDG uptake of the adrenal mass was greater than or equal to that of the liver. PET findings were interpreted as negative if the (18)F-FDG uptake of the adrenal mass was less than that of the liver. All studies were reviewed independently by 3 nuclear medicine physicians, and the results were then correlated with clinical follow-up or biopsy results when available. PET findings were positive in 71 adrenal masses. Sixty-seven of these were eventually considered to be metastatic adrenal disease. In the remaining 4, no changes in lesion size were noted on follow-up examinations. PET findings were negative in 42 adrenal masses, of which 37 eventually proved to be benign. Among the 5 adrenal masses that were false-negative, one was a large necrotic metastasis; 1 was a 2.4-cm lesion with central hemorrhaging, and the remaining 3 were lesions of less than 11 mm. The sensitivity, specificity, and accuracy for detecting metastatic disease were 93%, 90%, and 92%, respectively. (18)F-FDG PET is an accurate, noninvasive technique for differentiating benign from metastatic adrenal lesions detected on CT or MRI in patients with lung cancer. In addition, PET has the advantage of assessing the primary cancer sites and detecting other metastases.
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Our purpose was to evaluate the performance of (18)F-FDG PET/CT, using data from both the PET and the unenhanced CT portions of the study, in characterizing adrenal masses in oncology patients. One hundred seventy-five adrenal masses in 150 patients referred for (18)F-FDG PET/CT were assessed. Final diagnosis was based on histology (n = 6), imaging follow-up (n = 118) of 6-29 mo (mean, 14 mo), or morphologic imaging criteria (n = 51). Each adrenal mass was characterized by its size; its attenuation on CT, expressed by Hounsfield units (HU); and the intensity of (18)F-FDG uptake, expressed as standardized uptake value (SUV). Receiver operating characteristic curves were drawn to determine the optimal cutoff values of HU and SUV that would best discriminate between benign and malignant masses. When malignant lesions were compared with adenomas, PET data alone using an SUV cutoff of 3.1 yielded a sensitivity, specificity, positive predictive value, and negative predictive value of 98.5%, 92%, 89.3%, 98.9%, respectively. For combined PET/CT data, the sensitivity, specificity, positive predictive value, and negative predictive value were 100%, 98%, 97%, 100%, respectively. Specificity was significantly higher for PET/CT (P < 0.01). Fifty-one of the 175 masses were 1.5 cm or less in diameter. When a cutoff SUV of 3.1 was used for this group, (18)F-FDG PET/CT correctly classified all lesions. (18)F-FDG PET/CT improves the performance of (18)F-FDG PET alone in discriminating benign from malignant adrenal lesions in oncology patients.
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Adrenal myelolipomas are rare, benign mesenchymal tumors composed of mature adipose tissue and hematopoietic cells in varying proportions. Although the majority of cases occur as isolated adrenal lesions, myelolipomas have been described in association with various adrenal pathologic conditions. These conditions include enzyme deficiencies and hyperplastic and neoplastic lesions of the adrenal cortex, with perhaps endocrine dysfunction as a common feature. Ganglioneuroma is a benign tumor of the sympathetic nervous system that rarely produces symptoms of endocrine dysfunction. We report an unusual case of myelolipoma associated with ganglioneuroma of the adrenal medulla. The histogenesis of myelolipoma remains speculative. However, the close proximity to adrenal cortical cells within the stroma of ganglioneuroma suggests that the hormonal microenvironment may have played a role in the development of the myelolipoma.
Chapter
Historically, the adrenal tumor that was discovered incidentally, usually during an imaging procedure (CT, MRI, ultrasound) for symptoms unrelated to adrenal disease (e.g., back pain), was called an incidentaloma [1]. As more physicians (and patients on their own) ordered these easily available imaging studies for common diseases potentially related to adrenal pathology (and not the known syndromes), such as mild and nonparoxysmal hypertension, diffuse obesity, and diabetes, an increasing number of unsuspected (but hardly incidental) adrenal tumors were found. I have proposed that these tumors be included with the true incidentalomas under the broader term "adrenaloma" because they share the same diagnostic and therapeutic dilemmas [2]. The term adrenaloma implies that the discovered tumor (incidentally or not) arises from the adrenal but is not obviously an aldosteronoma, a Cushing's syndrome adenoma, a pheochromocytoma, a virilizing or feminizing tumor, or a functioning adrenal carcinoma. Recently, at a State of the Science Conference at the National Institute of Health, the term "clinically inapparent adrenal mass" was coined [3]. The widespread teaching is that most adrenalomas are indolent tumors, nonfunctioning and asymptomatic, causing no harm to the patient [4,5].Recent studies,however,have shown that a high percentage of these tumors can be subclinically functioning, causing symptoms milder than those encountered in the well known adrenal hyperfunctioning syndromes but still harmful to the patient [6-14]. Thus, the screening tests of serum potassium, urinary VMA and serum cortisol do not suffice and more detailed and in depth laboratory investigation is necessary.The fear of adrenal carcinoma that dictated the approach to these tumors in the past (with the main emphasis on the size of the tumor) should be changed to the fear of the subtle function of these usually benign adrenal cortical adenomas with coexistent metabolic pathology (e.g., hypertension, obesity, diabetes).
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Neuroblastoma represents one of the most challenging malignancies for treatment decisions because of its unusual biological behaviour. The features include spontaneous regression (regressive type), maturation to ganglioneuroma (maturative type) and largely treatment-resistant progression (progressive type). Current knowledge allows only partial prediction of type. For practical reasons, patients may be categorised as an ‘observation’, a ‘standard risk’ or a ‘high risk’ treatment arm. During the last 2 decades, 5-year survival rates for children with neuroblastoma have increased from 48 to 67%. The main achievements were the reduction of chemotherapy in patients with localised disease and the increased efficacy of chemotherapy in metastatic neuroblastoma stage 4 (5-year survival increased from 8 to 33%). Different goals for chemotherapy (e.g. stopping rapid progression, improvement of symptoms, induction and maintenance of remission) require different dosages and durations of treatment (range 1 week to 9 months). The main risks of chemotherapy are toxic death (rate up to 15%) predominantly during the periods of bone marrow depression and the development of secondary leukaemias (up to 7% cumulative risk after 4 years). In conclusion, the use of cytotoxic drugs can be completely omitted in a substantial proportion of low risk patients with neuroblastoma. On the other hand, for high risk patients with the disease, intensive polychemotherapy represents the basis and the backbone of treatment among other modalities.
Article
Ganglioneuromas (GNs) are neural crest cell-derived tumors and rarely occur in the adrenal gland. There are presently no markers that can reliably distinguish benign and malignant neuroendocrine tumors. Here we describe a 63-year-old woman who developed sudden chest pain and hypertension combined with increased stool frequency. An incidental adrenal mass 5 cm in size with a bright signal on T2-weighted magnetic resonance imaging was discovered. Biochemical evaluation and (131)I-metaiodobenzylguanidine (MIBG) scintigraphy were negative. Histopathological examination revealed a mature adrenal GN. Neuroblastoma, the immature form of a GN, is known for deletions on chromosomal locus 1p36, and adrenal tumors frequently show allele loss on 17p. To further elucidate the histo- and pathogenesis of adrenal GN, we performed loss of heterozygosity studies on chromosomal loci 1p34-36 and 17p13 (the p53 gene locus) after careful microdissection of tumor and normal tissue. We did not detect allelic losses at these loci with the informative polymorphic markers used, suggesting that these loci are not involved in tumorigenesis. In addition, immunohistochemical investigation of the GN was positive for vasoactive intestinal peptide, a hormone commonly expressed in ganglion cells. We suggest that in our patient with an adrenal GN, the combination of biochemical, scintigraphic, molecular, immunohistochemical, and histopathological findings are all consistent with the benign morphology of this tumor.
Article
Background: The ability to accurately assess tumor size is an important consideration during the preoperative evaluation of adrenal tumors, particularly solid nonfunctioning masses (incidentalomas or adrenalomas). Does the histological size of the adrenal tumor correspond to the preoperative computed tomography (CT)–estimated size?Objective: To evaluate the accuracy of CT in predicting the real size of adrenal tumors.Design: Retrospective review of all clinical records of patients who underwent adrenalectomy from 1984 through 1995. The mean tumor size reported from CT examinations was compared with the corresponding size obtained from the pathology reports and the statistical difference was evaluated.Setting University and private hospitals in Athens, Greece.Patients: Seventy-six patients who underwent adrenalectomy for various adrenal diseases and who met strict entry criteria.Results: For the entire population, the mean diameter of the tumors was estimated (CT reports) at 4.64 cm, but the real value (pathology reports) was 5.96 cm (P<.001). The underestimation held true for all of the studied subgroups that were defined by the different proposed cutoffs for malignancy. Three patients were incidentally found to have adrenal cancer, with the tumors measuring from 2.6 to 2.9 cm on CT. In addition, 4 pheochromocytomas were clinically and laboratory "silent" at the time of their discovery. The regression line (y=0.85 + 1.09χ) relating CT-estimated and histological tumor size was linear (r=0.90, P<.001).Conclusions: Computed tomography underestimates the real size of adrenal tumors. The CT-estimated value should be corrected accordingly to obtain the real size. The size of an adrenal tumor, even when corrected, cannot predict the tumor's clinical behavior in many cases. Surgeons should always cautiously interpret the proposed diagnostic cutoffs, especially when considering surgical or conservative management of small nonfunctioning adrenal tumors.Arch Surg. 1997;132:740-743
Article
Background: A case of retroperitoneal ganglioneuroma incidentally found by ultrasonography in a 56-year old woman is presented. Methods/Results: Computed tomography revealed a solid round tumor 6 cm in diameter on the upper pole of the right kidney. Iodine-131-metaiodobenzylguanidine weakly accumulated in the tumor 24 and 48 h after the injection. Her serum catecholamines were within normal limits apart from slightly elevated norepinephrine at one of the two examinations. During the operation her serum epinephrine and dopamine levels were elevated to about 50 and 800 times higher than preoperative values, respectively. Conclusion: This case is a rare ganglioneuroma in an adult patient that is endocrinologically active.
Article
A 65-year-old woman presenting with back pain, difficulties in walking and watery diarrhea. A right adrenal tumor and high excretion of catecholamines were found. Laboratory examinations showed raised levels of vasoactive intestinal polypeptide, pancreatic polypeptide, gastrin and calcitonin. Histology showed a combined pheochromocytoma—ganglioneuroma. The neoplastic cell population was immunohistochemically shown to contain tyrosine hydroxylase, neuropeptide Y, met-enkephalin, substance P, vasoactive intestinal polypeptide, calcitonin and calcitonin gene-related peptide. Postoperatively, the patient recovered fully and the hormone levels returned to normal.
Article
BACKGROUND Ganglioneuroma (GN) is considered by most to be a benign tumor of neural crest origin. It may evolve from differentiating neuroblastoma or may be diagnosed as primary ganglioneuroma. The rarity of this tumor and the lack of understanding of its biology often lead to inaccurate diagnosis and treatment.METHODS The authors analyzed clinical features and biologic behavior of primary ganglioneuroma in 49 patients who were registered with but were not part of the national neuroblastoma trials. Data included age and symptoms at diagnosis, gender, tumor localization and size, 123I-metaiodobenzylguanidine (mIBG) scintigraphy, secretion of catecholamines, histology, treatment, and outcome, whenever available.RESULTSPatients with primary ganglioneuroma were significantly older than patients with neuroblastoma. Median age at diagnosis was 79 months compared with 16 months (P < 0.0001). Ganglioneuroma were equally distributed between males and females (1.13:1). A preference of thoracic (41.5%) and abdominal, nonadrenal tumors (37.5%) was observed compared with adrenal GN (21%). At diagnosis, thoracic tumors appeared larger than nonthoracic ones. Local lymph node metastases occurred in two patients. One ganglioneuroma had metastasized to soft tissues. 123I-mIBG scintigraphy detected mIBG uptake at tumor site in 57% of the GN tumors. Levels of catecholamines in plasma and/or urine were increased in 39%. Slight immaturity of ganglion cells was observed in 93% of all ganglioneuroma tumors. None of the 22 tumors analyzed exhibited MYCN gene alterations. Although 12 patients had macroscopic residuals, no tumor progression or recurrence was observed in a median follow-up of 25 months.CONCLUSIONS Ganglioneuroma may present with metabolic activity such as increased secretion of catecholamines and/or mIBG uptake. There are no specific diagnostic signs or symptoms discriminating ganglioneuroma and neuroblastoma tumors. Therefore, ganglioneuroma requires tissue investigation for diagnosis. Prognosis after surgical resection without further therapy seems to be excellent. Cancer 2001;91:1905–13. © 2001 American Cancer Society.
Article
With rare exception, ganglioneuroma (GN) is a benign lesion which presents as a localized mass without metastatic potential and which is chemotherapy resistant. Thus, its distinction from neuroblastoma (NB) may be important. The diagnosis of GN implies the absence of neuroblastic elements. Incomplete resection prevents complete microscopic examination and raises the possibility that focal NB was not sampled. In an attempt to determine what features other than histology distinguish these two entities, we reviewed the charts of 25 patients with GN with regard to patient age and sex, tumor location and size, and urine catecholamine metabolite levels. One patient with GN (5%) and gross total resection had elevated quantitative vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels (2.4 × upper limit of normal for age), and two others had positive spot analyses for VMA. An additional patient with a large mass, multiple biopsies of which documented GN, also had greatly elevated (approximately 5 × normal) VMA and HVA levels. However, a subsequent attempt at resection disclosed several gross foci of NB. Even excluding this patient, there was a trend for elevated values in GN patients to correlate with tumor size (P = .07 and .14 for VMA and HVA, respectively). The incidence of elevated values appears to increase as a function of tumor size, and small tumors are not likely to result in positive urinary measurements. We conclude that while elevations of VMA and HVA are consistent with a well-documented diagnosis of GN, extreme elevations (>3 × nl) should prompt careful serial evaluation for occult NB.© 1994 Wiley-Liss, Inc.
Article
Current clinical teaching indicates that large (>5 cm in diameter) adrenal masses are often malignant. In a retrospective analysis of patients studied between 1977 and 1988 with computed tomography (CT), adrenal scintigraphy, and when available, magnetic resonance imaging (MRI) 45 were found to have adrenal masses greater than 5 cm (range 5–19 cm) in diameter. Thirty were benign (16 phaeochromocytomas, six adrenocortical adenomas, four adrenal cysts, two myelolipomas, an adrenal hematoma and a ganglioneuroma). Of 15 malignant masses, there were seven adrenocortical carcinomas, five adrenal metastases and three adrenal lymphomas. With the exception of the adrenal myelolipomas, cysts, and the ganglioneuroma neither CT nor MRI demonstrated sufficient diagnostic specificity to distinguish benign from malignant lesions. Functional scintigraphy with 131 I-6-Β-iodomethyl-19-norcholesterol for suspected adrenocortical lesions and 131 I-metaiodobenzylguanidine for suspected phaeochromocytomas frequently provided useful information. Peer Reviewed http://deepblue.lib.umich.edu/bitstream/2027.42/37953/1/1800780720_ftp.pdf
Article
To describe a patient with a virilizing adrenal ganglioneuroma and subclinical Cushing syndrome. Detailed clinical, laboratory, radiologic, and pathologic findings are presented, and the pertinent literature is reviewed. A 56-year-old postmenopausal woman was referred for evaluation of a 3.6- by 3.0-cm right adrenal mass, which had been diagnosed during a work-up for hirsutism. A bilateral oophorectomy done 2 months before the presentation failed to correct the elevated testosterone levels. On examination, she had severe hirsutism on her face, chest, back, and extremities, as well as male pattern baldness and clitoromegaly. Biochemical evaluation showed elevated total and free serum testosterone levels of 319 ng/dL (reference range, 20 to 70) and 78 pg/mL (reference range, 1 to 9), respectively, values in the adult male range. The serum dehydroepiandrosterone sulfate level was 117 microg/dL (reference range, 10 to 152), and the urine free cortisol was 10.4 microg/24 h (reference range, <45). A laparoscopic adrenalectomy revealed a 5.0-cm adrenal ganglioneuroma containing nests of adrenocortical cells. On the first day postoperatively, the serum cortisol level was <1.0 microg/dL. At 1 month after adrenalectomy, the total and free testosterone levels had declined to 16 ng/dL and 3.1 pg/mL, respectively. At 2 months postoperatively, normal results of a cosyntropin stimulation test (basal and peak cortisol levels of 13.6 and 20.0 microg/dL, respectively) indicated recovery of the hypothalamic-pituitary-adrenal axis. To our knowledge, this is the first case report of a virilizing adrenal ganglioneuroma with this unique pathologic finding and concomitant subclinical Cushing syndrome.
Article
A variety of hormonally active tumors may induce masculinization in the female. The authors report a ganglioneuroma located in the adrenal gland, which elaborated sufficient testosterone to virilize a woman.
Article
We report a case of a malignant peripheral nerve sheath tumor arising from a retroperitoneal ganglioneuroma in an adult male. There was no known previous history of a childhood neuroblastoma or of radiation therapy. In addition, the patient had no stigmata or family history of von Recklinghausen's neurofibromatosis. This case provides evidence that, although rare, spontaneous malignant transformation of ganglioneuroma can occur. Immunohistochemical studies supported that the spindle cell component was of nerve sheath origin.
Article
The successful application of [131I]metaiodobenzylguanidine (MIBG) in diagnosis and therapy of pheochromocytoma has led to its use in other tumors which derive from the neural crest and potentially concentrate this radiopharmaceutical as well. In the present series, [131]MIBG total-body scintigraphy was used for detection of neuroblastoma in 47 patients and 47 cases of other neural crest tumors. The method was found to be as reliable in neuroblastoma (sensitivity 95%, specificity 100%), as it is in pheochromocytoma. Although other neural crest tumors may concentrate [131I]MIBG, this is not a consistent finding; however, it is useful to investigate which tumors do, as this may provide an alternative treatment modality for some patients. Although followup is still very short, preliminary results of therapeutic use of [131I] MIBG in 21 patients indicate that this treatment modality may be effective in neuroblastoma and malignant pheochromocytoma.
Article
A 65-year-old woman presenting with back pain, difficulties in walking and watery diarrhea. A right adrenal tumor and high excretion of catecholamines were found. Laboratory examinations showed raised levels of vasoactive intestinal polypeptide, pancreatic polypeptide, gastrin and calcitonin. Histology showed a combined pheochromocytoma-ganglioneuroma. The neoplastic cell population was immunohistochemically shown to contain tyrosine hydroxylase, neuropeptide Y, met-enkephalin, substance P, vasoactive intestinal polypeptide, calcitonin and calcitonin gene-related peptide. Postoperatively, the patient recovered fully and the hormone levels returned to normal.
Article
A 58-year-old woman was virilized by a testosterone-secreting adrenal ganglioneuroma containing Leydig cells with crystalloids of Reinke. Examination of the ovaries revealed stromal hyperthecosis and hilus cell hyperplasia. The evidence from this case, as well as related cases in the literature, suggests that cells of the adrenal gland may become transformed into cells of gonadal type in both hyperplastic and neoplastic disorders.
Article
An adrenal medullary ganglioneuroma containing Leydig cells and revealed by a virilizing syndrome in a 56-year old woman is presented. The syndrome, associating with masculinization an elevated serum testosterone level and a normal urinary 17 ketosteroids, is uncommon in adrenal tumours. The tumour was located by computed tomographic (CT) scan and treated by right adrenalectomy. Microscopic examination showed typical features of an adrenal medullary ganglioneuroma containing Leydig cells with Reinke crystalloids. Positive immunohistochemical study confirmed the testosterone secreting nature of the cells. The presence of Leydig cells in the adrenal gland is discussed according embryogenic studies: 1. the common origin of the gonad and the adrenal cortex from the coelomic epithelium, 2. the vicinity of adrenocortical gland and gonad during embryogenesis, 3. the thecal metaplasia of mesenchymal cells, 4. the development of Leydig cells from Schwann cells. Fourth case published in the world literature.
Article
An unusual pheochromocytoma was incidentally discovered in a 48-year-old woman. The patient had a 3-year history of myasthenia gravis. At the time of examination in our hospital, the right adrenal tumor was incidentally discovered by ultrasonography of the abdomen. She had no history of headache, perspiration, palpitation or hypertension. Although blood catecholamine levels were within the normal limits, urinary secretion of catecholamine was elevated. Histologically, the tumor was diagnosed to be mixed ganglioneuroma/pheochromocytoma and histochemically confirmed to produce vasoactive intestinal polypeptide. Such a tumor is quite rare.
Article
To determine the radiologic appearance of ganglioneuromas of the adrenal gland and extra-adrenal retroperitoneum. A retrospective review was performed of imaging findings in nine patients with ganglioneuroma, two with ganglioneuroma-pheochromocytoma, and two with malignant peripheral nerve sheath tumor (PNST) that arose in a ganglioneuroma. Patients underwent computed tomography (CT) (n = 13), magnetic resonance (MR) imaging (n = 5), and ultrasonography (US) (n = 5). In nine patients with ganglioneuroma, the mass ranged in size from 2 x 4 x 4 cm to 21 x 21 x 22 cm. Imaging findings included attenuation entirely or predominantly less than that of muscle on contrast material-enhanced CT images (n = 9); heterogeneous, predominantly high signal intensity on T2-weighted MR images (n = 5); partial or complete surrounding of one or more major blood vessels with little or no luminal narrowing (n = 4) and calcifications (n = 4) on CT scans; and homogeneous, moderate echogenicity (n = 3) on US scans. CT findings in two patients with ganglioneuroma-pheochromocytoma included central necrosis (n = 1) and calcifications (n = 1). In the two patients with malignant PNST that arose in a ganglioneuroma, contrast-enhanced CT showed a large, markedly heterogeneous mass. Diagnosis of ganglioneuroma should be considered when typical CT or MR imaging findings are present. Findings in cases of ganglioneuroma-pheochromocytoma and malignant PNST arising in a ganglioneuroma appear to be nonspecific.
Article
Objective: The purpose of this study was to characterize the appearance of adrenal ganglioneuroma on CT scans and to define the specific imaging features of the tumor. Conclusion: Adrenal ganglioneuroma is an uncommon benign tumor that is revealed as a solid adrenal mass on CT scans. Specific CT features such as calcification and lesion enhancement may simulate a primary adrenal carcinoma. However, in our four patients, no signs of local invasion or vascular extension were noted. The specific diagnosis of adrenal ganglioneuroma requires either biopsy or surgical removal for documentation.
Article
The ability to accurately assess tumor size is an important consideration during the preoperative evaluation of adrenal tumors, particularly solid nonfunctioning masses (incidentalomas or adrenalomas). Does the histological size of the adrenal tumor correspond to the preoperative computed tomography (CT)-estimated size? To evaluate the accuracy of CT in predicting the real size of adrenal tumors. Retrospective review of all clinical records of patients who underwent adrenalectomy from 1984 through 1995. The mean tumor size reported from CT examinations was compared with the corresponding size obtained from the pathology reports and the statistical difference was evaluated. University and private hospitals in Athens, Greece. Seventy-six patients who underwent adrenalectomy for various adrenal diseases and who met strict entry criteria. For the entire population, the mean diameter of the tumors was estimated (CT reports) at 4.64 cm, but the real value (pathology reports) was 5.96 cm (P < .001). The underestimation held true for all of the studied subgroups that were defined by the different proposed cutoffs for malignancy. Three patients were incidentally found to have adrenal cancer, with the tumors measuring from 2.6 to 2.9 cm on CT. In addition, 4 pheochromocytomas were clinically and laboratory "silent" at the time of their discovery. The regression line (y = 0.85 + 1.09x) relating CT-estimated and histological tumor size was linear (r = 0.90, P < .001). Computed tomography underestimates the real size of adrenal tumors. The CT-estimated value should be corrected accordingly to obtain the real size. The size of an adrenal tumor, even when corrected, cannot predict the tumor's clinical behavior in many cases. Surgeons should always cautiously interpret the proposed diagnostic cutoffs, especially when considering surgical or conservative management of small nonfunctioning adrenal tumors.
Article
Size of adrenal mass is an important consideration during adrenal surgery, with regard to the choice of surgical approach and management of adrenaloma. The aim of the present study was to evaluate the precision of preoperative computed tomography (CT) on the size estimation of adrenal lesions and to review its potential implications on surgical decision making. Records of 110 patients who underwent adrenalectomy for various adrenal pathologies from 1981 to 1997 were reviewed retrospectively. Patients (n = 92) who had documented dimensions of adrenal mass by both preoperative CT and pathological examination were selected for analysis. CT overall underestimated the actual size of adrenal lesions by 16 per cent compared with measurement of resected specimens (mean 3.1 versus 3.6 cm; P < 0.001). There was a significant underestimation of the actual size of adrenal lesions measuring less than 6 cm by CT but not for lesions larger than 6.0 cm. Phaeochromocytoma (n = 17) was the only pathological condition consistently underestimated by CT. Conventional CT overall underestimates the real size of adrenal lesions, in particular phaeochromocytoma and adrenal tumours of less than 6.0 cm in size. Surgical decision making based on size should take this limitation into account.
Article
149 patients with adrenal incidentalomas were examined. Sixty-eight cases were histologically confirmed, five of them had ganglioneuromas. On the basis of these patients history current knowledge of this benign tumour was summarized. Histological and pathological characteristics of one tumour suggest that ganglioneuromas may develop by maturing of malignant neuroblastic tumours. The clinical symptoms (abdominal pain, meteorism) were local. In 2 of 5 cases mildly elevated levels of urinary vanillylmandelic acid and catecholamine could be measured. One patient had persisting hypertension after surgery. In an other patient previous diarrhoea stopped after the removal of tumour. On the basis of ultrasound and computertomographic features, the size and origin of a tumour and its relation to the surrounding organs can be well characterized. One patient was inoperable because of an infiltratively spreading tumour, but during five years of follow-up no tumour progression could be observed with computertomography. After surgery we could follow only 2 of 4 patients. Until now no recurrence of tumour were detected.
Article
To assess the sensitivity of positron emission tomography (PET) with 2-[fluorine-18]fluoro-2-deoxy-D-glucose (FDG) in pheochromocytomas and, secondarily, to compare images obtained with FDG PET to those obtained with metaiodobenzylguanidine (MIBG) scintigraphy. Twenty-nine patients with one or more known or subsequently proved pheochromocytomas underwent FDG PET (35 scans) and MIBG scintigraphy (35 scans). Tumor uptake of FDG was quantified on positive PET scans. Tumor uptake of FDG was detected in 22 of 29 patients. Most benign (seven of 12 patients) and most malignant (15 of 17 patients) pheochromocytomas and their metastases avidly concentrated FDG. In four patients whose pheochromocytomas failed to accumulate MIBG, uptake of FDG in the tumors was intense. For the majority of the 16 patients whose tumors concentrated both agents, however, ratings for MIBG images compared to FDG PET images for delineation of the tumor in comparison to background and normal organ accumulation were superior for nine patients (56%) and as good or better for 14 (88%). Most pheochromocytomas accumulate FDG. Uptake is found in a greater percentage of malignant than benign pheochromocytomas. FDG PET is especially useful in defining the distribution of those pheochromocytomas that fail to concentrate MIBG.
Article
Neuroblastoma represents one of the most challenging malignancies for treatment decisions because of its unusual biological behaviour. The features include spontaneous regression (regressive type), maturation to ganglioneuroma (maturative type) and largely treatment-resistant progression (progressive type). Current knowledge allows only partial prediction of type. For practical reasons, patients may be categorised as an 'observation', a 'standard risk' or a 'high risk' treatment arm. During the last 2 decades, 5-year survival rates for children with neuroblastoma have increased from 48 to 67%. The main achievements were the reduction of chemotherapy in patients with localised disease and the increased efficacy of chemotherapy in metastatic neuroblastoma stage 4 (5-year survival increased from 8 to 33%). Different goals for chemotherapy (e.g. stopping rapid progression, improvement of symptoms, induction and maintenance of remission) require different dosages and durations of treatment (range 1 week to 9 months). The main risks of chemotherapy are toxic death (rate up to 15%) predominantly during the periods of bone marrow depression and the development of secondary leukaemias (up to 7% cumulative risk after 4 years). In conclusion, the use of cytotoxic drugs can be completely omitted in a substantial proportion of low risk patients with neuroblastoma. On the other hand, for high risk patients with the disease, intensive polychemotherapy represents the basis and the backbone of treatment among other modalities.
Article
Adrenal ganglioneuromas are rare tumors originating from the neural crest tissue of the sympathetic nervous system. The clinical presentation of most patients is asymptomatic, and most of these tumors are hormone silent. Usually the tumor is detected incidentally by abdominal imaging studies for unrelated reasons. Herein, we review our cases of adrenal ganglioneuroma over the past 20 years. All patients diagnosed with primary adrenal ganglioneuroma between 1979 and 1999 were reviewed. There were 5 cases of pathologically confirmed adrenal ganglioneuroma. All patients underwent surgical excision of the adrenal tumors. The mean age of the 5 patients (3 women and 2 men) was 27 years (range, 11 to 45 years). The average diameter of the tumor was 8.2 cm (range, 6 to 10 cm). The mean follow-up period was 33.6 months (range, 3 to 92 months). One case involved mesenteric lymph nodes. This patient had persistent hypertension following the operation, but no evidence of tumor recurrence was noted over the 7-year follow-up period. The prognosis was excellent after surgical excision in our series. Awareness of this rare entity may add to the differential diagnoses of adrenal masses preoperatively.
Article
Ganglioneuroma (GN) is considered by most to be a benign tumor of neural crest origin. It may evolve from differentiating neuroblastoma or may be diagnosed as primary ganglioneuroma. The rarity of this tumor and the lack of understanding of its biology often lead to inaccurate diagnosis and treatment. The authors analyzed clinical features and biologic behavior of primary ganglioneuroma in 49 patients who were registered with but were not part of the national neuroblastoma trials. Data included age and symptoms at diagnosis, gender, tumor localization and size, (123)I-metaiodobenzylguanidine (mIBG) scintigraphy, secretion of catecholamines, histology, treatment, and outcome, whenever available. Patients with primary ganglioneuroma were significantly older than patients with neuroblastoma. Median age at diagnosis was 79 months compared with 16 months (P < 0.0001). Ganglioneuroma were equally distributed between males and females (1.13:1). A preference of thoracic (41.5%) and abdominal, nonadrenal tumors (37.5%) was observed compared with adrenal GN (21%). At diagnosis, thoracic tumors appeared larger than nonthoracic ones. Local lymph node metastases occurred in two patients. One ganglioneuroma had metastasized to soft tissues. (123)I-mIBG scintigraphy detected mIBG uptake at tumor site in 57% of the GN tumors. Levels of catecholamines in plasma and/or urine were increased in 39%. Slight immaturity of ganglion cells was observed in 93% of all ganglioneuroma tumors. None of the 22 tumors analyzed exhibited MYCN gene alterations. Although 12 patients had macroscopic residuals, no tumor progression or recurrence was observed in a median follow-up of 25 months. Ganglioneuroma may present with metabolic activity such as increased secretion of catecholamines and/or mIBG uptake. There are no specific diagnostic signs or symptoms discriminating ganglioneuroma and neuroblastoma tumors. Therefore, ganglioneuroma requires tissue investigation for diagnosis. Prognosis after surgical resection without further therapy seems to be excellent.