Adrenomedullary Hyperplasia in a Patient with Poorly Controlled Hypertension and Neurofibromatosis Type 1: A Case Report

A 73-year-old male with a known diagnosis of neurofibromatosis type 1 (or Von Recklinghausen’s disease) was referred to the authors’ unit because of suspected pheochromocytoma. He had poorly controlled hypertension with elevated metanephrine levels and incomplete cortisol suppression at 1 mg overnight dexamethasone test. Adrenal CT showed bilateral adrenal hyperplasia and a nodule suspected of being a pheochromocytoma, while 123I-MIBG SPECTCT showed intense hyperfixation in the left adrenal gland. Left transperitoneal Laparoscopic adrenalectomy was performed and the histologic diagnosis was diffuse and nodular adrenal medullary hyperplasia. We believe that medullary adrenal Hyperplasia is, among the causes of secondary hypertension, a separate entity and should be considered as a possible diagnosis in all patients with poorly controlled hypertension and radiological findings not typical of adenoma.