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Abstract
We describe a female adult patient who presented with acute retention of urine and vague abdominal discomfort. A provisional diagnosis of ovarian tumour was made after cross-sectional imaging. At laparotomy a very large retroperitoneal mass was biopsied and found to be a schwannoma after pathological examination. The clinical, radiological, and pathological features of this disease are discussed in this report.
... Retroperitoneal ancient schwannoma is an extremely rare tumor, which accounts for only about 0.5% to 12% of all retroperitoneal neoplasms (6). Etiology of schwannoma is unknown, but mostly found in peripheral nerve sheath in the limbs, head, and neck (7). Ancient schwannoma, in the retroperitoneal area, occurs more often between 40 and 60 years of age, with a slight tendency with male/female ratio of 2:3 (5). ...
... Malignancy is usually characterized by mitosis, pleomorphism, and blood vessel infiltration. Therefore, accurate monitoring is necessary after removal of retroperitoneal ancient schwannomas (1,3,7). ...
... Complete surgical resection is the treatment of choice for schwannoma and prognosis is excellent (7). Local recurrence is rarely seen and malignant transformation is extremely rare. ...
Background: Ancient schwannoma is an uncommon neoplasm originating from the peripheral nerve sheaths. Ancient schwannoma occurs mostly in head and neck region and its occurrence is very rare in retroperitoneal (about 0.7% to 2.6% of all schwannomas), and despite its benign nature, it can show malignant behaviors and it may be misdiagnosed with sarcomas in some cases. There is nonspecific clinical symptoms and radiologic findings. Evaluation of histology and immunochemistry is necessary for diagnosis, and complete surgical resection is the treatment of choice for schwannoma. Case Report: we report a unique case of retroperitoneal ancient schwannoma in a 50-year-old man with complaint of severe pain in the right flank area for 3 days ago, which was diagnosed with ancient schwannoma after evaluation of the histopathologic and immunohistochemical findings. After surgery, the patient was kept under regular follow-up since last 15 months without any evidence of recurrence.
... El término "Ancient schwannoma" fue acuñado por Ackerman y Taylor en 1951, para esta forma infrecuente de schwannoma; (5)(6)8,12,14) un subtipo histológico con escasas células tumorales, rodeadas por una matriz hialina, más rasgos de degeneración, asociada a una masa de lento crecimiento; (5,(6)(7)(8)(11)(12)14,16,(20)(21) esto podría sugerir malignidad, (1, 3,5,8,11,13,[16][17][18][19] y solo la falta de mitosis aboga por benignidad (5,(22)(23) . Se considera que, cambios degenerativos, formación de quistes, hemorragias, calcificación o hialinización, lleva a clasificarlos como schwannoma antiguo, (5,12) también ciertos cambios nucleares, pleomorfismo, lobulación, hipercromasia, encontrados en estos tumores. ...
... Se encuentra entre los 20 a 50 años (2,3,5,(7)(8)12,15,(17)(18)22,26,28) o poco más (11,16,27) y solo 5% se diagnostican en la niñez (2,15) . Se refieren más frecuentemente en mujeres (3,5,8,(10)(11)(12)(21)(22) , siendo la relación mujer/hombre de 3:2 (5,8,12,24) . ...
... Nuestro paciente era varón, de 37 años. Solo un trabajo de autores británicos (7) y otro de chinos (18) refieren predominancia de varones sobre mujeres, en la bibliografía consultada. ...
We present a patient with retroperitoneal ancient Schwannoma, who underwent surgery by the authors. Aspects of Schwannomas and the particular characteristics of the ancient Schwannomas are analyzed. The possibility of being related to von Recklinghausen's disease is analyzed, as well as the surgical possibilities, with emphasis on complete resection. The rare frequency of this tumor in the retroperitoneum is analyzed.
... Schwannomas, also called Neurilemmomas, are types of peripheral nerve tumors, benign disease, mostly located in the head and the neck, which are arising from Schwann cells of the nerve sheath. These tumors contribute to 0.75-2.6% of all retroperitoneal neoplasms, and the majority of patients are between 20 and 50 years old with a female predominance [1,2,6]. These tumors appear to arise in the retroperitoneum involving usually the paravertebral space or presacral region [3]. ...
... Schwannomas are benign and rare entities of peripheral nerve sheath origin with predominance usually in young to middle-aged adults, rarely degenerate into a malignant form [1,4]. These neurogenic tumors can be found anywhere but most commonly found in the upper extremities or in cranial and peripheral nerves in the head and neck [6]. They usually affect two women as often as men [8]. ...
Schwannomas are benign and rare entities of peripheral nerve sheath origin. The clinical presentation depends the size of tumor, and they may cause symptoms of abdominal pain, urinary difficulties, anemia, hematuria, and abdominal mass as a result of the pressure of the tumor to the adjacent structures. The diagnosis can only be established by immunohistochemical study. Complete surgical clearance remains the mainstay of treatment.
We report the case of a 35-year-old female patient consulted for chronic abdominal pain. Abdominal ultrasound and abdomino-pelvic CT scan identified a well-delineated, heterogenous retroperitoneal mass developed in anteraortocaval region measuring 55 × 65 × 88 mm. The lesion seemed to repress the inferior vena cava, and enhanced with contrast administration. The histopathological panel confirmed the diagnosis of retroperitoneal schwannoma.
... Schwannomas originate at peripheral nerve sheaths [1]. Those that occur in the retroperitoneal cavity are rare, accounting for only 0.75-2.6% of all retroperitoneal tumors [2,3]. Schwannomas are mostly benign and are associated with no characteristic clinical findings. ...
... Schwannomas occur only rarely in the retroperitoneal cavity and are predominantly benign. Previous reports indicate that schwannomas appear at incidences of 44.8% in the head and neck, 19.1% in the upper limbs, 13.5% in the lower limbs [1] and 0.75-2.6% in the retroperitoneum [2,3]. Retroperitoneal schwannomas are often huge because they produce no characteristic symptoms and become apparent only when they become large enough to produce compression symptoms such as abdominal pain and abdominal discomfort [4,5]. ...
Schwannomas that occur in the retroperitoneal cavity are rare. We herein report a patient who underwent safe laparoscopic resection by using a preoperative 3D computed tomography (CT) image and a fluorescent ureteral stent during the surgery.
A 47-year-old man presented with left lower abdominal pain. CT showed a 10-cm continuous retroperitoneal tumor originating at the third lumbar nerve in the lower left abdomen. Schwannoma was suspected. We underwent laparoscopic resection of the tumor guided by 3D images obtained preoperatively. A fluorescent ureteral stent was implanted during the surgery to improve visibility and protect the left ureter. The resection was completed without injury of other organs and vessels. The patient was discharged on postoperative Day 5.
By performing a preoperative simulation using 3D CT images, we could anticipate the anatomical findings and easily identify them intraoperatively. In addition, the fluorescent ureteral stent provided visual support, thereby contributing to safe surgery.
... The malignant peripheral nerve sheath tumours (MPNST) is defined as any malignant tumour differentiating into or deriving from the peripheral nerve sheath cells, with nonspecific symptoms, having high risk of recurrence locally and distant metastasis. 5,8 MPNST is particularly rare, affecting general population with an incidence of 0.001% and extremely rarespindle cell sarcoma in children, accounting for approximately 5-10% of nonrhabdomyosarcoma soft tissue sarcomas. 8,9 Schwannoma are tumours that are arise from the Schwann cells of peripheral nerve fibre and are generally found in the head, neck and extremities. ...
... 5,8 MPNST is particularly rare, affecting general population with an incidence of 0.001% and extremely rarespindle cell sarcoma in children, accounting for approximately 5-10% of nonrhabdomyosarcoma soft tissue sarcomas. 8,9 Schwannoma are tumours that are arise from the Schwann cells of peripheral nerve fibre and are generally found in the head, neck and extremities. 10 Retroperitoneal schwannoma are more commonly present in the paravertebral spaces and pre sacral region. ...
Schwannomas are benign nerve sheath tumors. Malignant schwannomas have been reported in literature but they frequently originate from the conversion of plexiform neurofibromatosis rather from the malignant degeneration of a benign schwannoma. Large retroperitoneal schwannomas are extremely rare tumours. They usually reach a large size when they arise retroperitoneally, which might raise suspicion for malignancy. Such tumors predominate in women schwannomas are usually solitary, slow growing and non- aggressive neoplasm usually found in head and neck region. Retroperitoneal schwannomas are usually large size are rare and are difficult to diagnose clinically. Nevertheless, the mainstay of management remains surgical removal and alternatively, the radio-therapy may prove to be effective. This case report of a 55 yrs male is based on such a patient in whom the diagnosis was established with great difficulty and intraoperative findings were something we had never encountered before.
... Nevertheless, reported clinical presentations of RSs are numerous and extremely variable, and include colitis, adrenal mass, psoas abscess, and acute urine retention. 1,10,11,12 Although referred pain and neurological deficits in the lower extremities caused by RSs have also been described, this presentation is rare. 13 Additionally, a clinician rarely suspects RS because the occurrence of unspecific lower back pain in middle-aged patients is almost always regarded as an epidemic symptom affecting the majority of the population once or more during life. ...
... 23 However, although the prognosis of benign RSs is good and local resection is generally sufficient, the incidence of tumour recurrence because of incomplete excision is 5% to 10%, and metastatic cases after surgery have been reported. 12,24 Therefore, these patients require long-term follow-up. Additionally, special care must be taken in patients with underlying Von Recklinghausen's disease in whom malignant transformation (neurofibrosarcoma), although rare, is usually observed. ...
Schwannomas are tumours that arise from Schwann cells of the peripheral nerve sheath and rarely occur in the retroperitoneum. We report a 45-year-old woman who presented with a 2-year history of continuous progressive right-sided lower back and dull flank pain radiating into her posterolateral thigh. Abdominal magnetic resonance imaging showed a homogenous soft-tissue tumour with thick capsular lining, which lay in the right retroperitoneum. The tumour was removed at surgery. A histological examination confirmed the diagnosis of benign encapsulated cellular schwannoma. Complete tumour excision should be regarded as the treatment of choice for benign retroperitoneal schwannomas. Successful treatment of these tumours requires thorough preoperative planning and a multidisciplinary approach.
... occurs in the retroperitoneum (2,6). They usually affect adult patients aged 20 to 50 years (7,8). ...
... Microscopically, they demonstrate Antoni A areas (wellorganised spindle cells in a palisade pattern) and Antoni B areas (less cellular, loose pleomorphic cells with an abundant myxoid component). Being positive for S-100 and negative for CD-34 are another two features supporting a correct diagnosis (3,8). In our case, the tumor had Antoni A dominated areas that were S-100 positive, but CD-34 negative (Figure 3). ...
Schwannomas are benign tumors originating from Schwann cells of the neural sheaths. Only 0.3% to 3% of the schwannomas are located the retroperitoneal space. Most schwannomas are asymptomatic and often found incidentally. The ideal treatment is complete surgical excision. Here, we report a case of a 49-year-old woman with retroperitoneal schwannoma.
... A 1.7% of all the reported RS are malignant [4]. They are mostly asymptomatic when found; however, larger tumors may cause abdominal pain or obstructive symptoms. ...
... RS are extremely rare tumors that arise from the Schwann cell of the sympathetic or parasympathetic nerves [4]. RS are seemingly important in the differential diagnosis of pancreatic/peripancreatic cystic lesions yet its preoperative diagnosis can be challenging. ...
... Schwannoma in retroperitoneum is a rare presentation. [1] Its symptoms depend on the size and area of tumor and are mainly due to compression of adjacent structures. Diagnosis depends on ultrasound, contrast enhanced computed tomography (CECT) and MRI and is aided by guided biopsy. ...
... Primary retroperitoneal tumors are themselves rare and schwannomas comprise only 0.5-1.2%. [1] Presentation of retroperitoneal schwannoma is non-specific and in most of the cases is due to compression of the adjacent structures. Abdominal ultrasonogram is a simple and non-invasive method for early detection of these tumors. ...
... Among all schwannomas, only 0.7% of benign ones and 1.7% of malignant ones are reported to be located in the retroperitoneal region. The majority of retroperitoneal schwannomas are benign in nature although malignant ones have also been reported [1][2][3]. ...
... Aggressive surgery is not indicated for benign retroperitoneal schwannomas. Although local resection is generally enough, metastatic cases have been reported after resection [1]. Therefore, followup is important for this patient. ...
Introduction. Schwannomas are quiet rare in the retroperitoneal region. Here, we describe an incidentally detected retroperitoneal schwannoma in the abdominal computerized tomography (CT) of a patient with acute appendicitis.
Case Presentation. A 38-year-old woman was admitted to the emergency service with the complaints of progressive abdominal pain and nausea for the last 24 hours. Abdominal examination was compatible with acute abdomen. Acute appendicitis was diagnosed by CT. During CT evaluation, a round shaped soft-tissue mass at the retroperitoneal area inferior to the right kidney was detected, The mass was resected and histology revealed schwannoma.
Conclusion. Rare tumoral lesions with benign course such as schwannoma can be detected incidentally.
... Affected sites include the spine, inner ear, gastro-intestinal tract, and mediastinum: most commonly affecting the inner ear. There is a predilection in the second and third decades of life and they are more common in women (Wong et al., 2010). Pelvic sites are rare but have been reported in the non-pregnant and pregnant state (Nithya et al., 2017). ...
Ancient schwannomas are benign tumours arising from the neural sheath of peripheral, cranial and autonomic nerves. They are commonly situated in the inner ear and spine with pelvic manifestations being rare. We present the case of a 30-year-old patient, who presented with an abdominal mass. MRI imaging suggested a broad ligament fibroid and open surgery was undertaken to remove it. Subsequent histology confirmed an ancient schwannoma. This case report details the rarity of such a condition and the need for a high index of suspicion as well as outlining management options and surveillance.
... Schwannomas originate from Schwann cells of the peripheral nerve fibers and are usually located in the head, neck, and flexor aspects of the extremities [1]. The majority of retroperitoneal schwannomas are benign in nature although malignant ones have also been reported [2]. The rarity of a retroperitoneal schwannoma merits its recording for the benefit of clinicians to consider this as a differential while assessment of a retroperitoneal mass. ...
... They rarely occur in the retroperitoneal spinal nerve roots; however, retroperitoneal tumors are the most common neurogenic tumors. [3] The diagnosis and treatment of schwannomas in the lumbar spinal canal has been reported in a large number of studies, while giant paravertebral schwannomas near the lumbar nerve roots with bone destruction are reported less frequently and can be easily misdiagnosed or missed entirely. ...
Rationale:
Schwannomas grow slowly, originating from the Schwann cells of the nerve sheath. Schwannomas of cranial origin have the highest incidence, followed by intraspinal schwannomas. However, paravertebral schwannoma is rare, and to our knowledge, giant paravertebral schwannomas near the lumbar nerve roots with bone destruction are extremely rare.
Patient concerns:
A 47-year-old Chinese woman complained of lower back soreness and a sensation of a bulging lumbar disc with no obvious cause for the past 3 years.
Diagnosis:
Lumbar magnetic resonance imaging showed a large mass with uneven density, 17 × 12 × 15 cm in size, located to the right of the 4th lumbar with obvious bony destruction. Histopathology and immunohistochemistry confirmed that this mass was a benign schwannoma.
Interventions:
Complete resection of the tumor (measuring about 17 × 12 × 15 cm in size) and vertebral reconstruction using internal fixation were performed.
Outcomes:
The patient was discharged without complications after surgery. The 3-year follow-up revealed that the patient recovered well with no evidence of recurrence.
Lessons:
Here, we emphasize the importance of careful radiological examination and reflect on the difficulty of tumor resection. Furthermore, understanding the treatment and diagnosis of lumbar paravertebral schwannoma is critical for plastic surgeons and radiologists when encountering similar cases.
... Schwannomas are nerve sheath tumors that usually affect the head, neck, and the flexor surfaces of the extremities. However, schwannoma in the retroperitoneal cavity is rare, accounting for about 0.75%-2.6% of all schwannomas, and for 4% of all retroperitoneal tumor [1][2][3] . Histologically, schwannomas are distinguished by the presence of areas of high and low cellularity called Antoni A and Antoni B tissue, respectively [4] . ...
Schwannoma in the retroperitoneal space is rare, and it is extremely rare in patients with no history of neurofibromatosis. We present a case of giant retroperitoneal schwannoma in a 52-year-old man who did not have neurofibromatosis. Because malignant transformation would be extremely rare in this circumstance, close imaging follow-up could avert the necessity for complete resection. The possibility of schwannoma should be considered when evaluating retroperitoneal tumors with the characteristic findings, even if there is no connection between the tumor and the intervertebral foramina.
... Although target and fasicular signs are characteristic radiological features of schwannomas in CT, they are not frequently seen in retroperitoneal schwannomas. 7 They appears as well demarcated, hypodense, homogeneous masses. Areas of heterogenicity are noted in case of tumor necrosis. ...
Solitary nerve sheath tumor such as Benign schwannomas arising in the pelvic retro peritoneum is infrequently reported. Retroperitoneal location accounts for 0.3-3.2% of primary schwannomas. We report a case of benign retroperitoneal pelvic schwannoma in pregnancy that was incidentally diagnosed when it presented with Preterm premature rupture of membranes and mechanical obstruction for labour. She underwent caesarean section and delivered a healthy baby. She was evaluated in the postoperative period by computerized tomography (CT) imaging studies and CT guided fine needle aspiration cytology (FNAC) was not diagnostic. Complete surgical excision of the tumor was achieved in the postpartum period. The adjacent vascular and urinary channels sustained no injuries and she had no neurologic deficit. Histology revealed spindle cell neoplasm composed of interlacing fascicles and sheets of spindle cell with focal areas of nuclear palisading and thick walled blood vessels. Immunohistochemistry was positive for S 100 suggesting schwannoma. Retroperitoneal location of schwannomas is rare and surgery is curative. Prognosis is good, since recurrence is rare.
... 6 CT could not differentiate schwannoma from ovarian in our case report, which may be due to absence of certain characteristic radiological signs of schwannoma in retroperitoneal location, such as target and fascicle signs. 18 CT guided biopsy and FNAC are not very promising for pre-operative diagnosis. 19 Having diagnosed, the recommended treatment is complete surgical excision. ...
Ovarian tumor is the most common diagnosis in postmenopausal women, presenting with a pelvic mass, though other surgical causes can still be a remote possibility. We report an extremely rare case of a woman, post hysterectomy, presenting with a pelvic mass where both clinical and radiological evaluations were suggestive of an ovarian tumor. The patient underwent laparotomy and a pre-sacral cystic mass was found and excised. Histopathology revealed a schwannoma as the final diagnosis. Hence, it mandates a high index of suspicion to consider other surgical possibilities, vigilant management in the form of advanced radiological evaluation, especially when dealing with doubtful cases of adnexal tumors prior to surgery.
... Primary retroperitoneal tumours are an extremely rare group of tumours, of which less than one third are benign and schwannoma represents the majority of them [1,3]. ...
Schwannomas are usually benign tumours arising from Schwann cells of peripheral nerve sheath. Retroperitoneal location is extremely rare compromising 0.5–5% of all schwannomas, except in patients having von Recklinghausen’s disease, in whom retroperitoneal location is more frequent and malignant cases are known to occur. Complete surgical excision with negative margins is the definitive treatment on which the diagnosis is usually made due to nonspecific radiologic appearance.
In this article we would like to present an unusual cause of recurrent, abdominal pain in an eighty-six-year-old mal
... Among all schwannomas, only 0.7% of benign ones and 1.7% of malignant ones are reported to be located in the retroperitoneal region. The majority of retroperitoneal schwannomas are benign in nature although malignant ones have also been reported ]. [1][2][3] Schwannomas are usually seen in adult population between the ages of 20 and 50. Symptomatology of benign schwannomas is highly nonspecific and depends on the location and size of the lesion. ...
... The differential diagnosis for retroperitoneal schwannomas includes other neurogenic tumors such as paraganglioma and pheochromacytoma as well as, liposarcoma and malignant fibrous histiocytoma. In addition to those, if the retroperitoneal schwannoma contains considerable amount of cystic degeneration, retroperitoneal cystic masses such as hematoma and lymphangioma should also be included in the diagnostic checklist 14 . ...
p>A 45 years old woman admitted to our Hospital with pain in the right flank and right hypochondrium. Her abdominal USG and CT scan showed a large complex cystic mass present in the deep soft tissue compressing the right kidney. Multiple gall bladder stones were also found in imaging. Both the adrenal glands and left kidney had a normal appearance. The mass was completely resected with extended right subcostal incision along with excision of Gall bladder. Histopathological examination of the specimen revealed a degenerated schwannoma. Patient was discharged on 8th day after operation without any complications.
J Bangladesh Coll Phys Surg 2015; 33(4): 225-228</p
... Histologically, schwannomas can be distinguished by the presence of areas of high and low cellularity, termed Antoni A and B tissue patterns, respectively (18,21,22). Microscopically, schwannomas demonstrate Antoni A areas (densely cellular, arranged in short bundles or interlacing fascicles) and Antoni B areas (fewer cells, organized, with great myxoid component) (28). Histopathological staining results showing positivity for S100 (9,21,22) and vimentin (29), and negativity for CD34 (9) are the most accurate markers for a final diagnosis. ...
Schwannomas rarely occur in the retroperitoneum, and are normally not aggressive. Preoperative diagnosis is difficult and the surgeon may confront blood vessels, nerves or organs damage, since the intra-operative findings are various. The diagnosis and treatment of malignant schwannomas in the retroperitoneum are even more challenging. In addition, the prognosis of malignant schwannomas is extremely poor. The present study reports the case of a 52-year-old woman who presented with a 6-month history of an abdominal mass in the left lower quadrant. The local doctor determined a preliminary diagnosis of abdominal giant tumor and referred the patient to the First Affiliated Hospital, Medical School of Xi’an Jiaotong University, (Xi’an, China). Following discussion, the patient underwent a surgical resection. Low-grade malignant schwannoma was diagnosed following histological examination. No evidence of recurrence or any other complication was observed at the 18-month follow-up examination. The present study reports a case of giant retroperitoneal schwannoma (RS), and includes a literature review in order to provide an overview of the diagnosis, treatment and prognosis of RS and discuss preoperative management strategies for the disease.
... They usually affect adult patients in the age group of 20-50 years and are more common in males. (3) The usual location is in head, neck and on flexor surfaces of extremities, retroperitoneum being the rare site with the incidence being 0.3-3.2%. Retroperitoneal schwannomas account for 1-10% of all primary retroperitoneal tumors.(1) ...
Schwannomas are rare tumors which arise from nerve sheath and are mostly benign in nature. They are usually located in the head, neck and flexor surfaces of extremities. Schwannomas are very rare in the retroperitoneal region. Amongst all schwannomas 0.7% of benign ones and 1.7% of malignant ones are located in the retroperitoneum. Preoperative diagnosis is difficult because of vague symptoms. We report a case of retroperitonal schwannoma in a 70 years female patient because of its rarity and unusual location.
... Schwannomas (neurilemmomas) are neurogenic benign tumors arising from schwann cells of peripheral nerve sheaths. While schwannomas commonly appear in head, neck, and extremities, they are quite rare in the retroperitoneal region, so that just 0.7% of benign and 1.7% of malignant types are observed in this region [1][2][3]. Schwannomas have a slow and prolonged clinical course before the diagnosis. Malignant transformations are uncommon in these types of tumors. ...
Retroperitoneal schwannomas are quite rare tumors. Isolated renal hydatid cyst is also rare, and it forms 2-4% of hydatid disease. Because of their infrequent occurrence, nonspecific signs and symptoms, and lack of distinguishing radiologic features, we report herein a case of right retroperitoneal mass in a 26-year-old woman which was found to be benign schwannoma following a percutaneous core needle biopsy and a large cortical cyst in the lower pole of the left kidney which was diagnosed as isolated renal hydatid cyst following exploration.
... Although "target" and "fascicular" signs are characteristic radiologic (CT) features of a schwannoma, these are not frequently seen in retroperitoneal schwannoma. 8 Another feature is the destruction of adjacent osseous structures, the exact pathogenesis of which is still unknown. Retroperitoneal schwannomas show cystic degeneration in up to 60% of cases, whereas calcification is seen in 23% of cases. ...
Schwannomas or neurilemomas are rare, benign tumors that originate from the neural sheath. A 54-year-old man presented with left flank discomfort and lumbar pain for 6 months. A computed tomographic scan confirmed the existence of a 12 × 9 cm heterogeneous retroperitoneal mass adherent to the left psoas muscle that displaced the left kidney anteriorly. Total laparoscopic removal of the tumor was performed and pathologic examination revealed a benign schwannoma.
Background:
Schwannomas are rare tumours that pose a significant management challenge in the abdomen, retroperitoneum and pelvis. No data are available to inform management strategy.
Methods:
A collaborative international cohort study, across specialist sarcoma units, was conducted to include adults presenting between 2000 and 2017 with histopathologically confirmed schwannomas within the abdomen, retroperitoneum or pelvis.
Results:
Of 485 patients across 12 centres, 38 (7·8 per cent) were discharged without follow-up, 199 (41·0 per cent) underwent early resection and 248 (51·1 per cent) had radiological monitoring. Of these 248 patients, 96 (38·7 per cent) eventually had surgery, giving an overall resection rate of 60·8 per cent (295 of 485). At baseline, median tumour volume was 90·1 (i.q.r. 26·5-262·0) cm3 . The estimated growth rate was 10·5 (95 per cent c.i. 9·4 to 11·6) per cent per year, and was consistent in the short term (within 2 years of diagnosis) and long term (beyond 2 years) (ρ = 0·405, P = 0·021). A decision to operate was more common in symptomatic patients (P < 0·001) and for rapidly growing tumours (growth rate more than 20 per cent per year) (P = 0·025). R0/R1 resection was achieved in 91·6 per cent of patients (263 of 287). Kaplan-Meier long-term recurrence rates after R0/R1 resection were 2·3 and 6·7 per cent at 3 and 5 years respectively.
Conclusion:
Specific recommendations include: indications for early surgery, prediction of growth from radiological monitoring, promotion of selective submacroscopic resection and cessation of postoperative imaging surveillance.
The presacral space is the site of a group of heterogeneous and rare tumors that are often indolent and produce nonspecific symptoms. Here we report a case of large multiloculated cystic mass in 53-year-old male presented with right-sided low backache. Provisional diagnosis of presacral schwannoma was made with the help computed tomography (CT) study and diagnosis was confirmed by histopathology.
Schwannomas are benign tumours that arise from Schwann cells of nerve fibres. They commonly occur in the head, neck, mediastinum
and extremities but pelvic occurrence is rare. We report a rare case of retroperitoneal tumour with Gynaec presentation. A 26-year-old
parous woman was admitted with abdominal mass, pain abdomen, painful menstruation and painful coitus for one year. Abdominal
examination revealed firm mass filling the lower abdomen with restricted mobility. On bimanual examination cervix was hitched against
pubic symphysis. Mass felt through anterior and left adnexa not tender, uterus felt close to the mass about 8-10 weeks size, right
adnexa free. USG revealed broad ligament fibroid with cystic right ovary. Hence myomectomy was planned, but intraoperatively it was
found that the mass was filling the pelvis close to bulky uterus with cystic right ovary. After informed consent while proceeding with
hysterectomy, necrotic, yellow colour material came out from the capsule like structure of the mass which was close to lower part of
posterior wall of uterus. Mass was removed except which was adherent to deeper structure left behind. Histopathological examination
revealed Schwannoma undergoing cystic degeneration. Since Schwannoma was mostly diagnosed incidentally, high degree suspicion
is necessary for diagnosis.
The occurrence of massive retroperitoneal schwannomasare extremely rare and their presence may be identified on cross sectional imaging or by insidious onset of non-specific and misleading symptoms with a predominance of lower back pain. They generally reach large proportions before producing such symptoms due to mass effect. Diagnosis is based on histopathologic examination and immunohistochemistry. Here we report the case of a large retroperitoneal mass in an 18 year old boy, that was excised in toto, which can sometimes be difficult due to the close proximity of the presacral venous plexus. © 2015, International Medical Sciences Academy. All rights reserved.
Schwannoma is the term given to tumours arising from Schwann cells of any nerve sheath. It may arise in the retroperitoneum, where it can attain enormous sizes and cause considerable diagnostic and therapeutic difficulties. A variety of incapacitating symptoms may arise, depending on its size alone and the related contagious organs.
A 71-year-old female, who was incapacitated by a giant abdominal mass, associated with weight loss, immobility, general weakness and constipation. Radiologically, the presence of a huge pelviabdominal tumour was confirmed. A preoperative tissue diagnosis was entertained but omitted, and we resorted to direct surgical excision instead. During surgery, significant bleeding from the surrounding lumbar vessels was encountered, but it was controlled and the tumour was excised intact. Histopathologically, it showed the histologic features of ancient schwannoma.
In the patient presented here, who was rendered immobile by the tumour, total excision or at least debulking seemed appropriate, regardless of any biopsy result.
The diagnosis of retroperitoneal schwannoma and its variant "ancient schwannoma" should be considered when a huge pelviabdominal tumour is encountered. Although CT guided biopsy may be helpful in reaching a preoperative diagnosis, this might not change the decision for the need of total tumour excision or at least debulking, in the presence of incapacitating symptoms. With large tumours, the possibility of perioperative exanginating haemorrhage should be remembered and the necessary precautions activated.
Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.
Schwannoma is a rare tumour arising from Schwann cells in myelinated neural sheath of the nerves. They can arise in various places
like head, neck, extremities, adrenal, retro peritoneum, psoas muscle. We here present a case of 33-year-old male who came with
features of acute pancreatitis, with incidentally detected mass in presacral region on ultrasonography. Investigations like CECT and MRI
were suggestive of neurofibroma, radical excision of the mass was done which showed solitary encapsulated tumour and cut sections
showed areas of necrosis, and histopathologically it was proven as schwannoma. Most of the schwannoma’s are benign, but rarely
malignant transformation can occur. It can be associated with von Recklinghausen disease. Benign tumours have excellent prognosis,
malignant tumours can present at later date with metastasis after excision also. Radiology plays a key role in diagnosis and follows up.
Treatment is radical excision of tumour; recurrence is most common and has to be carefully followed up.
Retroperitoneal schwannoma is a rare nerve sheath tumor; the surgical removal of this tumor is sometimes compromised by its location. The aim of this study is to analyze our experience with the diagnosis and treatment of this type of tumor.
We present our experience between 1999 and 2011 in the diagnosis and treatment of retroperitoneal schwannoma. During that time, we diagnosed and treated five female patients (four adults and one infant) with the condition. The tumors appeared sporadically and were not associated with neurofibromatosis or other syndromes. Diagnosis was performed by computed tomography (CT) imaging in four cases and by magnetic resonance imaging (MRI) in one case.
All patients underwent surgical treatment and complete resection of the lesion. An open resection was performed in four cases, and in the most recent case, the excision was conducted laparoscopically. In all of the cases, the histological diagnosis was retroperitoneal schwannoma, and in one case, there was a melanocytic variant that was not associated with Carney syndrome. At the time of this report, there has been no evidence of recurrence.
Retroperitoneal schwannoma is a tumor that is difficult to diagnose with imaging techniques, and because of its localization, the tumor is difficult to remove surgically.
Malignant schwannoma of the upper mediastinum originating from the vagus nerve is extremely rare.
A 46-year-old female was admitted for a left cervical mass which was associated with both hoarseness and Horner's syndrome. Chest computed tomography showed a mass extending from the left upper mediastinum to the left supraclavicular area. A fine needle aspiration cytological examination suggested primary lung cancer stage IIIB large cell carcinoma. After administering induction chemo-radiotherapy, a complete surgical resection was performed. The tumor was found to involve both the left vagus nerve and the left sympathetic nerve. Histological examination of the resected specimen revealed the tumor to be malignant schwannoma.
Despite incorrect preoperative diagnosis, the multimodality treatment administered in this case, including induction chemo-radiotherapy and surgery, proved to be effective.
Fourteen examples of an unusual mesenchymal tumor characterized by sheets and fascicles of mitotically inactive, hemosiderin-stippled, spindled, and pleomorphic cells, situated around an angiectatic vasculature, are described. The 14 tumours developed in eight women and six men (aged 32-83 years) and ranged in size from 2.3 to 8 cm. Eleven cases presented in the subcutaneous tissues, of which eight were located in the lower extremity. All featured prominent clusters of thin-walled ectatic vessels surrounded by perivascular hyaline material representing a combination of fibrin and collagen. In three cases the perivascular hyalinization was so extensive that it constituted more than half of the total tumor area. The tumor cells were similar to those of malignant fibrous hystiocytoma but differed from them by the presence of prominent intranuclear cytoplasmic inclusions, the extreme scarcity of mitotic figures, and the occasional presence of CD-34 expression. These tumors also shared several features with neurilemomas, such as their unusual vasculature, intranuclear cytoplasmic inclusions, lack of mitotic figures, and abundance of mast cells. They could be distinguished from neurilemomas, however, by the usual presence of infiltrative margins and the absence of S-100 protein. Follow-up information on eight patients (6 months to 25 years) indicated recurrences in four cases, with one of the three patients experiencing numerous recurrences over a 25-year period. No patient has developed metastases, however. We suggest that this tumor is a low-grade sarcoma of uncertain lineage in which the vascular changes are, in part, reflective of its slow growth.
To describe the imaging features of retroperitoneal and pelvic schwannomas.
The presenting cross-sectional imaging for 18 sequential patients with retroperitoneal or pelvic schwannomas was reviewed retrospectively. Note was made of tumour diameter, position, homogeneity, margin, shape, calcification and invasion into adjacent structures. Where MRI had been performed, T1 and T2 signal intensity relative to skeletal muscle, and the degree and pattern of enhancement with gadolinium, were also assessed.
Imaging from 13 patients was available for review. The mean tumour diameter was 8.7 cm (range 4 to 15 cm); 9 schwannomas were located in the pelvis and 4 in the retroperitoneum; 12 cases showed smooth, regular margins and 1 case irregular, invasive margins. The tumours were homogeneous in 5 cases and heterogeneous with cystic change in 8; in 2 cases there was smooth expansion of a sacral nerve root exit foramen, and in 1 there was bony destruction of the sacrum and extension of tumour into the spinal canal. In 5 cases MRI was performed; on T1-weighted images all tumours were isointense; on T2-weighted images 4 tumours were hyperintense and 1 was isointense to skeletal muscle. In all cases the diagnosis was confirmed by core biopsy.
Retroperitoneal and pelvic schwannomas typically form large, well-circumscribed masses in the retroperitoneum or presacral area, and frequently undergo cystic degeneration. They can occasionally cause bony changes in the spine, but otherwise do not invade or obstruct adjacent structures. Although they are rare, it is important for the radiologist to recognize the typical appearance of schwannomas because they can be mistaken for malignant tumours.
The aim of the study was to improve the diagnosis and treatment of retroperitoneal schwannoma by analysing clinical manifestations and postoperative course of this rare disease.
A retrospective analysis of 82 patients with retroperitoneal schwannoma between January 1951 and September 2004 was carried out.
The patients were 38 (46%) men and 44 (54%) women between the ages of 6 months and 70 years. The interval between clinical manifestation and diagnosis ranged from 10 days to 2 years. The main symptoms were abdominal distension (30.5%) and abdominal pain (20.7%). Only in 13 patients (15.9%) a correct preoperative diagnosis was made by either ultrasound-guided biopsy, computed tomography scanning or magnetic resonance imaging. All patients received operative therapy. Sixty patients (73.2%) underwent a total resection; 13 patients (15.9%) subtotal resection, but 9 patients (11.0%) had only an examination and a biopsy. Two patients (2.4%) had multiple schwannomas and two others had a simultaneous malignancy (adenocarcinoma of the ascending colon and squamous-cell carcinoma of the lung, respectively). Most of the retroperitoneal schwannomas were close to the spine. Pathological results showed 81 (98.8%) were benign schwannoma and 1 (1.2%) was a malignant one. The tumour size ranged from 3 to 22 cm. One benign schwannoma recurred 3 years after the operation. The patient with malignant schwannoma died 18 months after the operation because of metastasized disease.
Most of the retroperitoneal schwannomas are benign. It is difficult to make an accurate preoperative diagnosis. However, with the preoperative assessment of ultrasound-guided fine-needle aspiration, computed tomography and magnetic resonance imaging, the accuracy of diagnosis could definitely be improved. Treatment depends solely on surgery. Malignant schwannomas are insensitive to chemotherapy and radiation, resulting in poor prognosis.
Medcyclopaedia website: http://www.medcyclopaedia.com/library/topics
Neuroradiology: Neurilemmoma. Medcyclopaedia website:
http://www.medcyclopaedia.com/library/topics/volume_vi_
1/n/neurilemmoma. Accessed 1 May 2009.
Giant schwannoma in the pelvic retroperitoneum
- Lf Tauro
- C George
- A Kamath
- G Lobo
- P Shetty
- Br Hegde
Tauro LF, George C, Kamath A, Lobo G, Shetty P, Hegde
BR. Giant schwannoma in the pelvic retroperitoneum. J Clin
Diagn Res 2008;2:1210-4.