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Endocrine Abstracts (2022) 89 C28 | DOI: 10.1530/endoabs.89.C28

1Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, 10 Center Dr., Bldg. 10, Room 1E-3140, Bethesda, MD, 20892, USA; 2Radiology and Imaging Sciences, Warren Grant Magnuson Clinical Center, National Institutes of Health, 10 Center Dr., Bldg. 10, Bethesda, MD, 20892, USA; 3Nuclear Medicine Division, Radiology and Imaging Sciences, Warren Grant Magnuson Clinical Center, National Institutes of Health, 10 Center Dr., Bldg. 10, Bethesda, MD, 20892, USA; 4Positron Emission Tomography Department, Warren Grant Magnuson Clinical Center, National Institutes of Health, 10 Center Dr., Bldg. 10, Bethesda, MD, 20892, USA; 5Molecular Imaging Program, National Cancer Institute, National Institutes of Health, 10 Center Dr., Bldg. 10, Bethesda, MD, 20892, USA; 6Endocrine Oncology Branch, Center for Cancer Research, National Cancer Institute, 10 Center Dr., Bldg. 10, Room 4-5952, Bethesda, MD, 20892, USA; 7Department of Nuclear Medicine, La Timone University Hospital, CERIMED, Aix-Marseille University, Marseille, France; 8Nuclear Medicine, Radiology and Radiological Science, Johns Hopkins Medicine, Baltimore, MD, USA; 9Molecular Imaging and Therapy Service, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, MD, 10065, USA.


Background: Pheochromocytoma (PHEO) is a rare neuroendocrine tumor arising from chromaffin cells of adrenal gland that can cause life-threatening complications due to overproduction of catecholamines. Per EANM/SNMMI 2019 guidelines for radionuclide imaging of PHEO and paraganglioma, 18F-fluoro-L-dihydroxyphenylalanine (18F-FDOPA) is recommended as a positron emission tomography (PET) radiotracer of choice followed by 68Ga-DOTA(0)-Tyr(3)-octreotate (68Ga-DOTATATE) and 18F-fluoro-2-deoxy-D-glucose (18F-FDG), respectively in the detection of PHEO associated with multiple endocrine neoplasia 2A (MEN2A), caused by germline mutation in rearranged during transfection (RET) gene. No study has compared the diagnostic performance of these radiotracers in patients with MEN2A-related PHEO. The purpose of this prospective study was to evaluate and compare the detection rates of 18F-FDOPA, 68Ga-DOTATATE, 18F-FDG, and 18F-fluorodopamine (18F-FDA) PET or positron emission tomography/computed tomography (PET/CT), contrast-enhanced computed tomography (CT), and contrast-enhanced magnetic resonance imaging (MRI) in the detection of MEN2A-related PHEO.

Methods: Between 2008 and 2021, 19 patients (females:males, 10:9; mean age, 36.3±9.9 years) prospectively underwent 18F-FDOPA PET (n=3) or PET/CT (n=11), 68Ga-DOTATATE PET/CT (n=12), 18F-FDG PET/CT (n=18), and 18F-FDA PET (n=4) or PET/CT (n=4), CT (n=20) and MRI (n=18). The mean duration between scans was less than a month. The scans were evaluated by a nuclear medicine physician or radiologist. The histopathologic diagnosis served as the reference standard. The McNemar test was used to compare PHEO detection rates between the imaging modalities. Two-sided p values <0.05 were considered significant.

Results: Nineteen patients had 26 PHEOs [12 unilateral (7 right, 5 left) and 7 bilateral] on histopathology. 18F-FDOPA PET or PET/CT demonstrated a PHEO detection rate of 15/18 [83.3%, 95% confidence interval (CI): 58.6-96.4%]. 68Ga-DOTATATE PET/CT, 18F-FDG PET/CT, 18F-FDA PET or PET/CT, CT, and MRI showed PHEO detection rates of 12/15 (80.0%, 95% CI: 51.9-95.7%), 7/23 (30.4%, 95% CI: 13.2-52.9%), 6/12 (50.0%, 95% CI: 21.1-78.9%), 24/26 (92.3%, 95% CI: 74.9-99.1%), and 22/24 (91.7%, 95% CI: 73.0-99.0%), respectively. The difference in detection rates between 18F-FDG and other scans was significant (P<0.05).

Conclusions: The study was performed in a small cohort of MEN2A-related PHEO patients demonstrating CT, an anatomic imaging modality with the highest detection rate whereas in functional imaging, 18F-FDOPA PET/CT showed the highest detection rate followed by 68Ga-DOTATATE, 18F-FDA, and 18F-FDG in supporting the 2019 EANM/SNMMI guidelines. A difference in detection rates between various imaging modalities and 18F-FDG was found.

Abstract ID 21580

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