Publication: Adrenal cortex-sparing surgery for bilateral multiple pheochromocytomas in a patient with von hippel-lindau disease
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KU-Authors
KU Authors
Co-Authors
Acar, Ömer, Tefekli, Ahmet
Musaoğlu, Ahmet
Rozanes, İzzet
Emre, Ali
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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.
Source
Publisher
Hindawi Ltd
Subject
Medicine, General, Internal
Citation
Has Part
Source
Case Reports in Medicine
Book Series Title
Edition
DOI
10.1155/2012/659104