ArticleThe Journal of clinical endocrinology and metabolism2018
18F-FDOPA PET/CT Imaging of MAX-Related Pheochromocytoma.
Article in The Journal of clinical endocrinology and metabolism, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
25 citing papers in PubMed, 1 synthesis or guideline pooled it, 46 citations in OpenAlex.
- Multiple Endocrine Neoplasia Type 5 due to Germline MAX Mutations: A Systematic Review of Tumor Spectrum and Clinical Features.Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists · 2026Pooled it
- Genetic and Clinical Profiles of Pheochromocytoma and Paraganglioma: A Single Center Study.Frontiers in endocrinology · 2020Trial
- Genotype-dependent [European journal of nuclear medicine and molecular imaging · 2026Article
- Molecular imaging and radioligand therapies in pheochromocytomas and paragangliomas.Endocrine-related cancer · 2026Review
- CT features predict tumour invasion of adrenal pheochromocytoma: a retrospective observational study.BMC medical imaging · 2025Observational
- Approach to the Patient: Concept and Application of Targeted Radiotherapy in the Paraganglioma Patient.The Journal of clinical endocrinology and metabolism · 2024Review
- Imaging of Pheochromocytomas and Paragangliomas.Endocrine reviews · 2024Review
- Genotype and clinical phenotype characteristics ofFrontiers in endocrinology · 2024Article
- Case Series:Frontiers in endocrinology · 2024Article
- The Spectrum of Familial Pituitary Neuroendocrine Tumors.Endocrine pathology · 2023Review
- Pheochromocytoma: a changing perspective and current concepts.Therapeutic advances in endocrinology and metabolism · 2023Review
- Personalized Management of Pheochromocytoma and Paraganglioma.Endocrine reviews · 2022Review
- Overview of the 2022 WHO Classification of Familial Endocrine Tumor Syndromes.Endocrine pathology · 2022Review
- Imaging of Pheochromocytoma and Paraganglioma.Journal of nuclear medicine : official publication, Society of Nuclear Medicine · 2021Review
- A Clinical Challenge: Endocrine and Imaging Investigations of Adrenal Masses.Journal of nuclear medicine : official publication, Society of Nuclear Medicine · 2021Review
- Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force.Endocrinology and metabolism (Seoul, Korea) · 2021Article
- HEREDITARY ENDOCRINE TUMOURS: CURRENT STATE-OF-THE-ART AND RESEARCH OPPORTUNITIES: Metastatic pheochromocytomas and paragangliomas: proceedings of the MEN2019 workshop.Endocrine-related cancer · 2020Review
- Genetics, diagnosis, management and future directions of research of phaeochromocytoma and paraganglioma: a position statement and consensus of the Working Group on Endocrine Hypertension of the European Society of Hypertension.Journal of hypertension · 2020Review
- 18F-FDOPA PET/CT accurately identifies MEN1-associated pheochromocytoma.Endocrinology, diabetes & metabolism case reports · 2020Article
- Pheochromocytoma and Paraganglioma: From Clinical Findings to Diagnosis.Sisli Etfal Hastanesi tip bulteni · 2020Review
Corrections and comments
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Authors and funding
14 authors at 6 institutions in 2 countries.
Funding
Abstract
Context: MYC-associated factor X (MAX) has been recently described as a new susceptibility pheochromocytoma (PHEO) gene with a total of ~40 reported cases. At present, no study has specifically described the functional imaging phenotype of MAX-related PHEO. Objective, Patients, and Design: The objective of the present study was to present our experience with contrast-enhanced computed tomography (CT) and 18F-fluorodihydroxyphenylalanine (18F-FDOPA) positron emission tomography (PET)/CT in six consecutive patients (four at the initial diagnosis and two at the follow-up evaluation) with rare, but clinically important, MAX-related PHEOs. In five patients, 18F-FDOPA was also compared with other radiopharmaceutical agents. Results: The patients had five different mutations in the MAX gene that caused disruption of Max/Myc interaction and/or abolished interaction with DNA based on in silico analyses. All but one patient developed bilateral PHEOs during their lifetime. In all cases, 18F-FDOPA PET/CT accurately visualized PHEOs that were often multiple within the same gland or bilaterally and detected more adrenal and extra-adrenal lesions than did CT (per-lesion sensitivity, 90.9% vs 52.4% for CT/magnetic resonance imaging). The two PHEOs missed on 18F-FDOPA PET/CT were <1 cm, corresponding to nodular adrenomedullary hyperplasia. 68Ga-DOTA,Tyr3-octreotate PET/CT detected fewer lesions than did 18F-FDOPA PET/CT in one of three patients, and 18F-fluorodeoxyglucose PET/CT was only faintly positive in two of four patients with underestimation of extra-adrenal lesions in one patient. Conclusions: MAX-related PHEOs exhibit a marked 18F-FDOPA uptake, a finding that illustrates the common well-differentiated chromaffin pattern of PHEOs associated with activation of kinase signaling pathways. 18F-FDOPA PET/CT should be considered as the first-line functional imaging modality for diagnostic or follow-up evaluations for these patients.
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