Bilateral pheochromocytoma: Clinical characteristics, treatment and longitudinal follow‐up. (22nd June 2020)

• Record Type: Journal Article
• Authors: Kittah, Nana Esi; Gruber, Lucinda M.; Bancos, Irina; Hamidi, Oksana; Tamhane, Shrikant; Iñiguez‐Ariza, Nicole; Babovic‐Vuksanovic, Dusica; Thompson, Geoffrey B.; Lteif, Aida; Young, William F.; Erickson, Dana
• Is Part Of: Clinical endocrinology. Volume 93:Number 3(2020)
• Page Start: 288
• Page End: 295
• Publication Date: 2020-06-22
• Subjects: adrenal tumour -- Multiple endocrine neoplasia 2 -- neurofibromatosis‐1 -- pheochromocytoma -- von Hippel‐Lindau disease; Endocrinology -- Periodicals; 616.4005
• DOI: 10.1111/cen.14222
• Languages: English
• ISSNs: 0300-0664

Abstract

Abstract: Objective: Comprehensive data about patients with bilateral pheochromocytoma are 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 centre. Patients: All patients with bilateral pheochromocytoma evaluated at Mayo Clinic in Rochester, Minnesota between January 1951 and December 2015. Measurements: Tumour 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 tumours (80%). Median time to metachronous tumours 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 tumours 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 paediatric patients. Genetic disease was identified in 80% of patients, predominantly MEN2A. Multicentric tumours were common, but most were still cured following adrenalectomy.