Introduction: Ganglioneuromas are rare, benign tumors of mature ganglion cells, arising from the sympathetic ganglia and 20% are located in the adrenal medulla, representing 14% of adrenal incidentalomas. About 1/3 secrete catecholamines but hypertension and other adrenergic symptoms are rare. About 60% are depicted with 131I- MIBG.
Case report: A 72-year-old man was admitted for a left adrenal tumor 9×8 cm, incidentally found in abdominal CT scan for investigation of haematuria. The patient was normotensive and diabetic type II. Basic and dynamic tests for cortisol and aldosterone hypersecretion were normal, 24 h urinary normetanephrine (2070 μg (105354)), norepinephrine (422 μg (20100)) and dopamine (425 μg (80350)) were elevated (normetanephrine was 7 times the upper normal limit). Urinary epinephrine and metanephrine were normal. 131I- MIBG whole body scan showed an intense uptake by the mass. Serum CgA was increased (334 ng/ml (<98)). Serum calcitonin (121 pg/ml (<15)) was increased without ultrasound apparent thyroid nodules. The tumor was diagnosed as a pheochromocytoma and a left adrenalectomy was performed with no perioperative complications. Histology revealed a gaglioneuroma with mature ganglion cells and abundant Schwann cells. Immunohistochemical staining for calcitonin was positive. Six months postoperatively, urinary catecholamines and normetanephrines were normal, abdominal CT scan showed a normal right adrenal, 131I- MIBG scan was negative and serum CgA decreased to almost normal levels (102 ng/ml). Calcitonin also decreased to normal (4.6 pg/ml).
Conclusion: Untill now only pheochromocytomas and composite pheochromocytomas tumors containig both chromafine and ganglion cells are reported to secrete calcitonin. To our knowledge this is the first reported ganglioneuroma secreting calcitonin.