title: Adrenal cortex-sparing surgery for bilateral multiple pheochromocytomas in a patient with von hippel-lindau disease.
abstract: Pheochromocytomas can be a part of familial neoplastic syndromes, in which case they tend to be multiple and involve both adrenal glands. Therefore, sparing adrenocortical function represents a major concern while dealing with these hereditary lesions. Herein, we describe the clinical characteristics and the management strategy of a patient with von Hippel-Lindau (VHL) disease who had multiple, bilateral pheochromocytomas as well as bilateral renal masses, pancreatic masses, and a paracaval mass. Only a portion of the left adrenal gland has remained in situ after two consecutive open surgeries and a percutaneous radiofrequency ablation which have been performed to treat the various components of this syndrome. No adrenal or extra-adrenal pheochromocytoma recurrences have been detected during a follow-up period of more than 2 years. Pancreatic and adrenal functions were normal throughout the postoperative period and never necessitated any replacement therapy. Adrenal cortex-sparing surgery is a valid option for VHL disease patients who present with synchronous bilateral adrenal pheochromocytomas.
authors.affiliation: School of Medicine, Koç University, 34450 Istanbul, Turkey ; Department of Urology, VKF American Hospital, 34365 Istanbul, Turkey.
authors.forename: Tarık
authors.initials: T
authors.lastname: Esen
authors.forename: Omer
authors.initials: O
authors.lastname: Acar
authors.forename: Ahmet
authors.initials: A
authors.lastname: Tefekli
authors.forename: Ahmet
authors.initials: A
authors.lastname: Musaoğlu
authors.forename: Izzet
authors.initials: I
authors.lastname: Rozanes
authors.forename: Ali
authors.initials: A
authors.lastname: Emre