The significance of cystic adrenal lesions in multiple endocrine neoplasia IIB syndrome.

Cystic adrenal lesions are common, but cystic pheochromocytomas are rare. In the setting of a cystic adrenal mass in a patient with multiple endocrine neoplasia syndrome (MEN) IIB, the diagnosis of pheochromocytoma must be considered. We report a 29-year-old woman with typical phenotype of MEN IIB (marfanoid habitus, thick blubbery lips, mucosal ganglioneuromas) and a history of medullary thyroid carcinoma. She presented with headaches, palpitations and tremors. Computed tomography revealed a left cystic adrenal mass. The likelihood of the lesion being a pheochromocytoma was thought to be low due to its cystic appearance. However, urine ephinephrine and metanephrine levels were elevated. She underwent a left adrenalectomy and histological examination revealed a cystic pheochromocytoma.