The clinical spectrum of adrenal ganglioneuromas extends from severe hypertension to an asymptomatic incidentaloma; two cases and mini review of literature.

Abstract

Purpose: Herein, we present two interesting cases of adrenal ganglioneuromas (AGNs), namely, (a) a giant AGN which caused a severe episode of hypertension during a therapeutic dilation and curettage for early pregnancy loss, and (b) a case of a composite clinically silent adrenal ganglioneuroma-pheochromocytoma. Furthermore, we conducted a mini literature review on AGNs. CASE 1: A 31-year-old female patient presented with a history of early pregnancy loss at 9 ½ weeks. She underwent a therapeutic dilation and curettage during which she developed hypertension (~ 210/120 mm Hg). She reported no history of arterial hypertension, flushing, or tachycardia and her medical history was unremarkable. Further work-up, revealed a large heterogeneous tumor with microcalcifications in the left adrenal gland. Endocrine work-up was negative for hormonal excess. Magnetic resonance angiography (MRA) of the abdomen showed that the tumor was 'surrounding and strangling' the left renal vessels and the inferior vena cava. The patient successfully underwent an open left adrenalectomy and nephrectomy. Histology revealed an adrenal mature ganglioneuroma with Schwannian stroma being dominant. Her postoperative course was uneventful and she remains recurrence-free 6 years after surgery. CASE 2: A 37-year-old male patient was admitted to the hospital because of abdominal pain and hematuria. Computer tomography identified a 4-cm right adrenal lesion. Due to elevated urinary metanephrines, he underwent laparoscopic right adrenalectomy after appropriate alpha-blockade preparation preoperatively. Histology was consistent with pheochromocytoma with a component of ganglioneuroma, thus, a composite pheochromocytoma. His post-operative course was excellent and he remains asymptomatic and recurrence-free 60 months post-operatively.

Conclusion: These are two challenging cases of (a) a giant non-secreting AGN presenting with severe hypertension due to renal vessel compression and (b) a clinically silent composite pheochromocytoma-ganglioneuroma presenting with abdominal pain and hematuria. The size in the first case and the concurrence with a pheochromocytoma but a clinically silent phenotype in the second case highlight the fact that AGNs can be diagnostic chameleons.