Background: The ganglioneuroma (GN) is a benign tumor of the sympathetic nervous system following the sympathogonies that affects children and young adults. This is a rare tumor (7/1 000 000) which can be located along the sympathetic chain from the neck to the pelvis. In 20% of cases, the GN is localized in the adrenal.
Objective and hypotheses: Report observations of eight patients with GN.
Observations: Eight patients (two boys and six girls) with a mean age of 12 years (417) hospitalized for exploration discovered adrenal mass on ultrasound after the onset of abdominal pain (n: 8) associated with partial subocclusif syndrome in one case (patient aged 4 years old). clinical and laboratory exploration did not reveal hormonal hypersecretion. All tumors were large (≥6 cm), heterogeneous and invasives. Retroperitoneal localization was observed in three patients. The fixing MIBG scintigraphy was observed only in two cases Seen surgery was too risky because of the close relationship of the tumor with large vessels. Monitoring shall be decided in the younger, while other patients were referred for embolization (n: 5) or radiotherapy (n: 2).
Conclusion: GN preferentially affects children and adolescents. 3/5 of them occur before the age of 20. Women are more affected than men. They are voluminous tumors most often found incidentally during a radiological examination or a palpable mass. They can be revealed by non-specific pain or signs of compression. Some GN are hyperfunctioning (catecholamines, VIP, or testosterone) The diagnosis of GN is histopathological. The evolution of these tumors is slow and the prognosis is mainly due to mechanical complications of infectious or order related to tumor volume.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology