Familial Cushing Disease

Abstract

A 28-year-old woman and her niece presented with manifestations of the Cushing syndrome. Endocrine studies, including small-and large-dose dexamethasone-suppression test confirmed the diagnosis of pituitary ACTH excess. The first patient had a sella of normal size and went into clinical and hormonal remission after external pituitary irradiation, and she has remained well of follow-up for 9 years. The niece had an enlarged sella turcica on tomography and is receiving a course of external pituitary irradiation.