Endocrine Abstracts

Introduction: Ganglioneuromas (GNs) are rare, benign tumors, arising from neural crest cells. Their incidence amongst adrenal masses is 0.3%-6%. The majority are incidentalomas and hormonally inactive but a subset may cause compressive effects. Presentation in the context of genetic syndromes (NF1/NF2, RET, MAX, Von Hippel Lindau disease) has been reported. We present two cases of adrenal GNs: Case 1. A 31-year-old female patient who presented with a history of early pregnancy loss at 9 ½ weeks. During a therapeutic dilatation and curettage, she developed persistently high blood pressure. Further work-up showed a large heterogeneous tumor with microcalcifications in the left adrenal gland. It compressed the left renal veins and the inferior vena cava. Endocrine evaluation was negative for hormonal hypersecretion. The patient underwent an open left adrenalectomy and nephrectomy. Histology revealed a left adrenal mature ganglioneuroma with dominant schwannian stroma. Her postoperative course was uneventful and she remains asymptomatic and normotensive during a 50-month follow-up. Case 2. A 37-year-old male patient was admitted to the hospital because of left abdominal pain and haematuria. Computer tomography demonstrated a 4 cm right adrenal lesion. Despite that urine catecholamines were elevated the patient did not present arterial hypertension or flushing. After appropriate alpha and beta blockade he underwent laparoscopically a right adrenalectomy. Histology revealed a 4.5-cm mass with components of phaeochromocytoma and ganglioneuroma, thus a composite phaeochromocytoma. His post-operative course was excellent and he remains asymptomatic 60 months later.ConclusionWe describe two challenging cases of a) a giant adrenal GN that compressed the left renal vein and presented with hypertension and b) a composite phaeochromocytoma-ganglioneuroma that presented with abdominal pain and haematuria. The extension of ganglioneuroma in the first case and the concurrence with a clinically silent phaeochromocytoma in the second case, highlight the fact that adrenal GNs can be diagnostic chameleons.