Bilateral pheochromocytoma: Clinical characteristics, treatment and longitudinal follow‐up

Abstract

Objective Comprehensive data about patients with bilateral pheochromocytoma is limited. We aimed to describe the clinical presentation, genetic analysis, treatment, and outcomes of patients with bilateral pheochromocytoma. Design A retrospective study at a tertiary care center Patients All patients with bilateral pheochromocytoma evaluated at Mayo Clinic in Rochester, Minnesota between January 1951 and December 2015. Measurements tumor size, genetic testing, plasma/urine metanephrines and catecholamines Results A total of 94 patients (51% women) were diagnosed with bilateral pheochromocytoma at a median age at first presentation of 31 years (range, 4‐70). Bilateral disease was noted in 8.0% of pheochromocytoma patient overall and 37.5% of patients 18 years of younger. Most patients presented with synchronous tumors (80%). Median time to metachronous tumors was 4.5 years (range, 1‐38). Genetic disease was identified in 75 (80%) patients, including MEN 2A (42.6%), VHL (19.1%), MEN 2B (9.6%), and NF1 (8.5%). Excess catecholamines were present in 97% of patients. Patients with synchronous pheochromocytoma commonly underwent simultaneous bilateral adrenalectomy (99%), and 18 (24%) had cortical‐sparing surgery. Multicentric tumors were reported in 23 of 77 (30%) patients with available data. Recurrent disease was found in 9.6% of patients, and 8.5% developed metastatic disease. Median follow‐up was 8.5 years. At the study conclusion, 4 patients had died due to pheochromocytoma or adrenalectomy. Conclusions Bilateral pheochromocytoma occurred in 7.0% of adults with pheochromocytoma and 37.5% of pediatric patients. Genetic disease was identified in 80% of patients, predominantly MEN2A. Multicentric tumors were common, but most were still cured following adrenalectomy.