Hindawi Publishing Corporation Case Reports in Medicine Volume 2012, Article ID 659104, 5 pages doi:10.1155/2012/659104 Case Report Adrenal Cortex-Sparing Surgery for Bilateral Multiple Pheochromocytomas in a Patient with Von Hippel-Lindau Disease Tarık Esen,1, 2 O¨ mer Acar,2 Ahmet Tefekli,2 Ahmet Musaog˘lu,2 I˙zzet Rozanes,3 and Ali Emre4 1 School of Medicine, Ko¸c University, 34450 Istanbul, Turkey 2 Department of Urology, VKF American Hospital, 34365 Istanbul, Turkey 3 Department of Radiology, VKF American Hospital, 34365 Istanbul, Turkey 4 Department of General Surgery, VKF American Hospital, 34365 Istanbul, Turkey Correspondence should be addressed to O¨ mer Acar, omer acar @hotmail.com 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. 1. Introduction Adrenal surgery has gained popularity throughout the world most probably because of the increased radiologic detection of small, solid, asymptomatic adrenal masses. These inci- dentalomas are mostly small in size and nonfunctioning. However, surgery is needed either because of the size or the hormone-producing nature of the lesion, in at least 15% of the cases [1]. Adrenal surgery has been accepted as a total adrenal resection per deﬁnition. In a patient with unilateral disease and a healthy contralateral adrenal gland, this is mostly not a problem. Yet the radiologic presence of the contralateral gland is not necessarily an evidence for healthy function. Moreover, adrenal masses are common and mostly bilateral and/or multiple in patients with hereditary syndromes, where a bilateral adrenalectomy could have devastating side eﬀects either with the resulting adrenal insuﬃciency or the need for life-long hormonal replacement [2]. Recently, the concept of partial adrenalectomy is intro- duced for the above-mentioned reasons and prompted us to report a case where a staged cortex-sparing adrenal surgery was done in a patient with von Hippel-Lindau (VHL) disease and successfully eliminated multiple bilateral pheochromocytomas without causing adrenal insuﬃciency. 2. Case Report A 31-year-old, otherwise healthy, young male patient pre- sented with incidentally detected bilateral small solid renal and adrenal masses, a cystic mass at the head of the pancreas, and a paracaval mass. The patient deﬁned no relevant family history and had no obvious symptoms. Physical examination ﬁndings (including serial blood pressure measurements)