Ganglioneuroma of the Adrenal Gland: A Rare Tumor in a Rare Location

Division of Surgical Oncology, Department of General Surgery.
Case Reports in Oncology 09/2012; 5(3):487-9. DOI: 10.1159/000342445
Source: PubMed


A 62-year-old man presented to his general practitioner complaining of non-specific back pain. He underwent a computerized tomography scan and magnetic resonance imaging that revealed a large left adrenal mass. A thorough investigation of this mass revealed it to be a non-secreting tumor. At surgery, a large tumor of the left adrenal was found. The final pathology report revealed a ganglioneuroma of the adrenal gland.

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Available from: Hayim Gilshtein, Jun 20, 2014
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    ABSTRACT: Background. Ganglioneuromas (GNs) are neural crest cell-derived tumors and rarely occur in the adrenal gland. They are usually asymptomatic and hormonally silent. The majority of cases are detected incidentally during work-up for unrelated conditions. Hormone-secreting pure adrenal GNs in adults are extremely rare. To date, only four cases have been reported in the English literature. Case report. We describe an adult case of endocrinologically active adrenal GN incidentally diagnosed in a 64-year-old male patient with history of uncontrolled hypertension. On physical examination, he had a blood pressure (BP) of 160/100 mmHg. Abdominal computed tomography and magnetic resonance imaging showed a large solid tumor (8.5 × 7.5 × 7 cm) in the right adrenal gland. Urinary levels of norepinephrine, normetanephrine, vanillylmandelic acid and dopamin were elevated, although urinary level of epinephrine was suppressed. Right adrenalectomy was performed for treatment purposes. The histological diagnosis of the resected tumor was adrenal GN. Conclusions. Hormone-secreting pure adrenal GN occurs very rarely in adults and preoperative diagnosis is difficult. Adrenal GN may present with hormonal activity such as increased secretion of catecholamines and their metabolites. There are no specific diagnostic signs and symptoms discriminating GN and pheochromocytoma. Therefore, histopathological examination need for a definitive diagnosis of adrenal GN. The prognosis after completed surgical resection without further therapy seems to be excellent. To our knowledge, the present case is the second report that describes hormone-secreting pure adrenal GN in an adult from Turkey in the English literature. We discuss this case and review the literature on this unusual entity.
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    ABSTRACT: 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.
    Full-text · Article · Jan 2014 · Acta endocrinologica
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    ABSTRACT: Neuroblastoma (NB) is the most common extracranial solid tumor in children. Diarrheal NB is quite rare and is not easy to diagnose in the early stage. Six cases of diarrheal NB in our hospital treated from 1996 to 2006 were retrospectively analyzed, including characteristics such as electrolyte imbalance, pathologic features, vasoactive intestinal peptide (VIP) immunohistochemical staining results, treatment, and prognosis. All patients were boys with 3-8 loose or watery stools each day and routine fecal tests were normal. Abdominal tumors were identified by B-ultrasound. Drugs were ineffective. Three patients underwent surgery, and the remaining three patients received surgery and chemotherapy. Diarrhea stopped after treatment in five patients. Two patients died due to intractable hypokalemia. The tumor was located in the adrenal gland in four patients, in the upper retroperitoneum in one patient, and in the presacral area in one patient. Pathologic findings were NB and ganglioneuroblastoma. Five patients were at clinical stage I-II, and one was at stage III. Four patients survived (followed-up for 6 mo to 4 years). Immunohistochemical staining for VIP was positive. Refractory diarrhea is a paraneoplastic syndrome of NB and is rare. Patients aged 1-3 years who present with chronic intractable diarrhea should be followed closely. Intractable diarrhea, hypokalemia, and dysplasia are the initial clinical manifestations. Increased VIP is characteristic of this disease. Potassium supplementation plays a vital role in the treatment procedure, especially preoperatively. The prognosis of diarrheal NB is good following appropriate treatment.
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