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SAT-310 18F-FDOPA PET/CT Accurately Identifies Pheochromocytoma in 70-Year-Old Male with MEN1

Authors :
Lee S. Weinstein
Naris Nilubol
William F. Simonds
Roxanne Merkel
Karel Pacak
Craig Cochran
Abhishek Jha
Sunita K. Agarwal
James Welch
Jenny E Blau
Adel Mandl
Corina Millo
Aisha Tepede
Source :
Journal of the Endocrine Society
Publication Year :
2019
Publisher :
The Endocrine Society, 2019.

Abstract

18F-FDOPA PET/CT accurately identifies pheochromocytoma in 70-year-old male with MEN1 Background: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant inherited disorder that causes tumors most often in the parathyroid, pancreas, and pituitary. Pheochromocytoma in MEN1 is extremely rare (< 2% of cases). Identifying tumor functionality by imaging is challenging given the multiplicity of tumors in MEN1. Case Report: We are reporting a case of a 75-year old male first diagnosed at age 60 with MEN1. Sequencing of MEN1 revealed the pathogenic variant c.249_252delGTCT causing a frameshift mutation. At the time of diagnosis, he was found to have mild hyperparathyroidism status post 2-gland resection, non-functional pituitary microadenoma and bilateral adrenal masses (2.6 cm left; 2.4 cm right). The patient was normotensive and asymptomatic, with elevated plasma normetanephrine 114 pg/mL (upper limit of normal 112) and metanephrine 148 pg/mL (upper limit of normal 61). The remainder of his biochemical workup at that time was normal. The patient remained normotensive and asymptomatic, and routine yearly CT evaluation demonstrated growth of the left adrenal mass to 3.9 cm (9 Hounsfield unit (HU) pre-contrast, and post-contrast HU of 15), and right adrenal mass to 2.6 cm. Follow-up biochemical evaluation revealed an adrenergic biochemical phenotype with elevated plasma metanephrine 432 (12-61 pg/ml) and normetanephrine 291 (18-112 pg/ml) levels, with normal norepinephrine 198 (84-794 pg/ml), dopamine

Details

ISSN :
24721972
Volume :
3
Database :
OpenAIRE
Journal :
Journal of the Endocrine Society
Accession number :
edsair.doi.dedup.....97aff41f1e2eb5cf39c57caa45ee0e07
Full Text :
https://doi.org/10.1210/js.2019-sat-310