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

Abstract EN

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.