TY - JOUR
T1 - Bilateral pheochromocytoma
T2 - Clinical characteristics, treatment and longitudinal follow-up
AU - Kittah, Nana Esi
AU - Gruber, Lucinda M.
AU - Bancos, Irina
AU - Hamidi, Oksana
AU - Tamhane, Shrikant
AU - Iñiguez-Ariza, Nicole
AU - Babovic-Vuksanovic, Dusica
AU - Thompson, Geoffrey B.
AU - Lteif, Aida
AU - Young, William F.
AU - Erickson, Dana
N1 - Publisher Copyright:
© 2020 John Wiley & Sons Ltd
PY - 2020/9/1
Y1 - 2020/9/1
N2 - 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.
AB - 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.
KW - Multiple endocrine neoplasia 2
KW - adrenal tumour
KW - neurofibromatosis-1
KW - pheochromocytoma
KW - von Hippel-Lindau disease
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U2 - 10.1111/cen.14222
DO - 10.1111/cen.14222
M3 - Article
C2 - 32410303
AN - SCOPUS:85087169390
SN - 0300-0664
VL - 93
SP - 288
EP - 295
JO - Clinical Endocrinology
JF - Clinical Endocrinology
IS - 3
ER -