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

Nana Esi Kittah, Lucinda M. Gruber, Irina Bancos, Oksana Hamidi, Shrikant Tamhane, Nicole Iñiguez-Ariza, Dusica Babovic-Vuksanovic, Geoffrey B. Thompson, Aida Lteif, William F. Young, Dana Erickson

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)288-295
Number of pages8
JournalClinical Endocrinology
Volume93
Issue number3
DOIs
StatePublished - Sep 1 2020

Keywords

  • adrenal tumour
  • Multiple endocrine neoplasia 2
  • neurofibromatosis-1
  • pheochromocytoma
  • von Hippel-Lindau disease

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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