Hormone-secreting ganglioneuroma: A rare entity

Abstract

Ganglioneuromas are slow-growing benign counterparts of neuroblastoma groups along with ganglioneuroblastomas. Herein, we report a rare case of hormone-secreting benign ganglioneuroma in a 4-year-old kid. A 4-year-old male child was referred to our outdoor patient department from the primary health center to evaluate for the persistent complaint of increase in weight and raised blood pressure. On computed tomography (CT) of the abdomen and pelvis, an 6.4 cm × 6.5 cm mass lesion was seen over the right adrenal gland which was compressing segments VI and VII of the liver, and the right hilar region showed a 1.6 cm × 1.2 cm × 0.9 cm enlarged lymph node. The rest of the scan was clear. The patient was therefore started on cyclophosphamide and vincristine, received two cycles, and 4 weeks following it, a diagnostic CT scan of the abdomen, pelvis, and thorax was undertaken which showed a 5.1 cm × 4.5 cm × 6 cm lesion over the right suprarenal region abutting inferior vena cava, inferior surface of the liver, and superior renal surface. Surgical laparotomy followed by right adrenalectomy was done which on pathological evaluation to a surprise showed features suggestive of maturing ganglioneuroma with no evidence of any blastemal components. However, the posterior resection margin turned out to be positive. Radiation was omitted despite the margins being positive. Very few cases of hormone-secreting pediatric ganglioneuroma have been reported in literature and their management requires a meticulous approach.

Keywords

• benign
• ganglioneuroma
• hormone
• radiation
• surgery