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Case Reports in Medicine
Volume 2012, Article ID 659104, 5 pages
Case Report

Adrenal Cortex-Sparing Surgery for Bilateral Multiple Pheochromocytomas in a Patient with Von Hippel-Lindau Disease

1School of Medicine, Koç University, 34450 Istanbul, Turkey
2Department of Urology, VKF American Hospital, 34365 Istanbul, Turkey
3Department of Radiology, VKF American Hospital, 34365 Istanbul, Turkey
4Department of General Surgery, VKF American Hospital, 34365 Istanbul, Turkey

Received 22 June 2012; Revised 18 September 2012; Accepted 24 September 2012

Academic Editor: Chin-Jung Wang

Copyright © 2012 Tarık Esen et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.