18F-FDOPA PET/CT accurately identifies MEN1-associated pheochromocytoma

Abstract

Summary Pheochromocytoma (PHEO) in multiple endocrine neoplasia type 1 (MEN1) is extremely rare. The incidence is reported as less than 2%. We report a case of a 76-year-old male with familial MEN1 who was found to have unilateral PHEO. Although the patient was normotensive and asymptomatic, routine screening imaging with CT demonstrated bilateral adrenal masses. The left adrenal mass grew from 2.5 to 3.9 cm over 4 years with attenuation values of 9 Hounsfield units (HU) pre-contrast and 15 HU post-contrast washout. Laboratory evaluation demonstrated an adrenergic biochemical phenotype. Both 18F-fluorodeoxyglucose (18F-FDG) PET/CT and 123I-metaiodobenzylguanidine (123I-mIBG) scintigraphy demonstrated bilateral adrenal uptake. In contrast, 18F-fluorodihydroxyphenylalanine (18F-FDOPA) PET/CT demonstrated unilateral left adrenal uptake (28.7 standardized uptake value (SUV)) and physiologic right adrenal uptake. The patient underwent an uneventful left adrenalectomy with pathology consistent for PHEO. Post-operatively, he had biochemical normalization. A review of the literature suggests that adrenal tumors >2 cm may be at higher risk for pheochromocytoma in patients with MEN1. Despite a lack of symptoms related to catecholamine excess, enlarging adrenal nodules should be biochemically screened for PHEO. 18F-FDOPA PET/CT may be beneficial for localization in these patients. Learning points: 18F-FDOPA PET/CT is a beneficial imaging modality for identifying pheochromocytoma in MEN1 patients. Adrenal adenomas should undergo routine biochemical workup for PHEO in MEN1 and can have serious peri-operative complications if not recognized, given that MEN1 patients undergo frequent surgical interventions. MEN1 is implicated in the tumorigenesis of PHEO in this patient.

Individual approach in the treatment of prolactinoma in a patient with multiple endocrine neoplasia type 1 syndrome

“…Так, при синдроме множественной эндокринной неоплазии тип 1 (МЭН1) одним из наиболее частых клинических проявлений является развитие аденом гипофиза, в частности пролактином [6]. Для этого синдрома характерно также развитие опухолей паращитовидных желез (ПЩЖ), эндокринной части поджелудочной железы (ПЖ) и целого ряда других локализаций [7,8,9]. По данным исследований при МЭН1 гораздо чаще встречаются макропролактиномы, и эффективность консервативной терапии ниже [10,11,12].…”

Personalized molecular visualization and radiotheranostics of adrenal tumors

“…ФХ и ПГ, сочетающиеся с мутациями группы псевдогипоксии (SDHx, FH, VHL, EPASI) лучше обнаруживаются при ПЭТ/КТ с 68 Ga-DOTATAT [76], в то время как опухоли с мутациями группы киназ (RET, NF1, MAX, HRAS) успешнее визуализируются с помощью 18 F-FDOPA [76][77]. Согласно случаю, недавно представленному Tepede et al, ПЭТ/ КТ с 18 F-FDOPA является более чувствительным методом в диагностике ФХ с мутацией MEN1, в сравнении с 123 I-МЙБГ и 18 F-ФДГ [78]. Таким образом, 18 F-FDOPA обладает наибольшей чувствительностью для экстракортикальных ПГ (особенно для ПГ головы и шеи), симпатических ПГ и первичных ФХ.…”

Familial MEN1 Syndrome Diagnosed on Functional Imaging: A Case Report with Clinical and Genetic Correlation

“…1,2 The prevalence of MEN 1 syndrome is estimated to be 2 to 10 people per 1,00,000. 3,4 MEN1 syndrome is characterized by occurrence of parathyroid, pituitary, and pancreatic tumors. Additionally, some patients develop adrenocortical tumors, meningioma, lipoma, face angiofibromas, and collagenomas, as well as pheochromocytomas, although rarely.…”