Background: Ganglioneuromas (GN) are rare benign tumours arising from the neural crest cells and it is equally rare to find them arising from the adrenal gland. We report a case of a 10-year old boy with an incidentally identified adrenal GN.
Case report: A 10-year old boy had presented with the complaints of excessive weight gain noticed by the parents since the age of 2 years. There were no other complaints except for sleep apnea. A strong genetic history of obesity in both the parents and their families was present. As part of the workup for obesity, an ultrasound of abdomen was done and incidentally an adrenal tumor was found on the right side, measuring 6 cm × 3 cm. The findings were confirmed on Computerized Tomography of the adrenal gland. Hormonal tests were done to ascertain the type and origin of the tumour. Overnight Dexamethasone suppression test showed an 8:00am Cortisol level <1.0 μg/dl. Serum aldosterone and DHEAS were within normal range. Serum electrolytes were also normal. Thus, adrenal cortical tumours were excluded. 24 h Urinary VMA was 0.89 mg/day (Normal-Upto 15 mg/day). Urine metanephrine was slightly raised 154.05 μg/day (Normal 5113 μg/day), however plasma metanephrine was normal 38.60 pg/ml (Normal below 180 pg/ml). A MIBG Scan was done and delayed imaging at 72 h revealed moderate intense tracer concentration in the right suprarenal region corresponding to the lesion seen on CT scan, suggesting an MIBG avid neuroendocrine tissue. Keeping a close differential of phaeochromocytoma in mind, we decided to surgically remove the lesion after preoperative preparation with alpha and beta-blockers. A highly vascular tumour, measuring 6×4×5 cm of the right adrenal gland with adhesions to the IVC was found intraoperatively, and right adrenalectomy was performed. The patient had no intraoperative or postoperative complications. Histological examination revealed mature neural and ganglion cells, suggesting a mature subtype of GN. The patient on a 1year follow up remains stable and there is no recurrence.
Conclusion: GN may mimic other adrenal malignancies such as phaeochromocytoma. Careful evaluation with endocrine tests and imaging procedures are necessary for an accurate diagnosis. Definitive diagnosis is by histological examination. The prognosis is excellent following surgical removal.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology