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.

Figure 1

T1-weighted abdominal MRI in the axial plane demonstrating bilateral multiple renal masses of varying sizes and MR signal intensities.

Figure 2

T2-weighted MR image in the coronal plane demonstrating bilateral, adrenal masses and renal cysts. The large adrenal mass on the right side has almost completely replaced the adrenal tissue.

Figure 3

Arterial phase T1-weighted image in the axial plane following intravenous contrast depicts hypervascular adrenal masses on both sides and an approximately 3 cm hypervascular mass in the uncinate process of the pancreas consistent with a neuroendocrine tumor.

Figure 4

Abdominal MRI following two sessions of open surgery and radiofrequency ablation for a left-sided recurrent solid mass. There is no sign of recurrent or residual solid tumor in the kidneys. Remnant adrenal tissue on the left side was free of any recurrent lesion and was measuring 24.62 × 9.48 mm in diameter. Multiple cysts are visible in the right kidney.