Pheochromocytoma and paraganglioma in patient with extirpated ganglioneuroma
Ljiljana Marina, Miomira Ivovic, Svetlana Vujovic, Milina Tancic-Gajic, Vlada Zivaljevic, Zorana Arizanovic, Ana Nenezic & Dragan Micic
Ganglioneuromas are benign sympathetic tissue tumors originating from neural crest cells. A 40-year-old female patient, was admitted to our department for endocrinological evaluation of incidentally discovered tumor of right adrenal gland. For about a year before admission she had a pain under the right costal arch. An ultrasound and computed tomography (CT) showed 2 ovoid cystical tumors of right adrenal (34 and 32 mm, respectively). Three months prior admission she had several hypertensive crises with vertigo, with average blood pressure values of 120/80 mmHg. At 27 years of age she had a surgery because of a mediastinal tumor. Pathohistological (PH) finding confirmed ganglioneuroma. In family history her father, brother and a cousin suffered from heart disease and hypertension. Physical finding showed Horner Sy as a consequence of previous surgery. Careful endocrine testing showed very high levels of noradrenalin in urine, and metaiodobenzylguanidine (MIBG) scintigraphy of whole body showed accumulation in both adrenal glands. After preparation with phenoxybenzamine she underwent surgery and right adrenal gland was extirpated along with another tumor. PH confirmed pheochromocytoma of right adrenal and paraganglioma of the same side. Genetic testing showed presence of Von HippelLindau mutation Val84Met, and one of her two tested children was also positive. Postoperative testing showed high levels of catecholamines and CT scan revealed new tumor on the left side in front of abdominal aorta, left renal artery and left renal vein and behind splenal vein. After preparation with phenoxybenzamine she underwent another surgery, tumor was extirpated and PH confirmed paraganglioma. Levels of postoperative catecholamines showed to be normal.
In our case ganglioneuroma occurred in patient with VHL mutation whereas in literature they are usually found in patients with MEN2B.