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

Purpose . Herein, we present two interesting cases of adrenal ganglioneuromas: a) a giant adrenal ganglioneuroma, which provoked 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 adrenal ganglioneuromas. 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 underwent successfully an open left adrenalectomy and nephrectomy. Histology revealed an adrenal mature ganglioneuroma with schwannian stroma as dominant. Her postoperative course was uneventful and she remains recurrence-free six years after her surgery. Case 2. A 37-year-old male patient was admitted to the hospital because of abdominal pain and haematuria. 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 adrenal ganglioneuroma presenting with severe hypertension due to renal vessels’ 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 one, highlight the fact that adrenal ganglioneuromas can be diagnostic chameleons.