Bilateral pheochromocytoma: Clinical characteristics, treatment, and longitudinal follow-up.
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.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.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.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.