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.

Single Stage Bilateral Adrenalectomy (Cortical-Sparing) and Pancreatectomy (Corpus-Sparing) in a Patient with Von Hippel-Lindau Disease

“…Thus function-preserving surgical resections have been recommended due to low malignancy incidence especially for young patients to rescue them from the development of adrenal insufficiency, life-long steroid and hospital dependency in patients with VHL disease. [ 3 , 4 ]…”

Single-stage laparoscopic adrenalectomy for pheochromocytoma and enucleation of a pancreatic neuroendocrine tumor in Von Hippel-Lindau disease: A case report

“…Adrenal surgery in the past was understood as a total adrenal resection, creating problems and important side-effects with resulting adrenal insufficiency and/or the need of using hormone therapy for the remainder of the patient's life, particularly when the resection involved both glands (8). Consequently, the idea of partial adrenalectomy was introduced, trying to achieve a cortexsparing surgery (9). In the present case study, it was possible to perform adrenalectomy since the lesion was well demarcated, and a part of the normal gland remained at its upper pole.…”

Asian Conference on Tumor Ablation Guidelines for Adrenal Tumor Ablation

“…For this reason, adrenal ablation may provide a longer adrenal insufficiency-free survival period in hereditary recurrent adrenal tumors compared to adrenalectomy ( Figs. 1 , 2 ) [ 54 , 55 ].…”