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.
03 - 07 May 2008
European Society of Endocrinology