Multiple Endocrine Neoplasia Type 5 due to Germline MAX Mutations: A Systematic Review of Tumor Spectrum and Clinical Features
Authors' conclusion
Our systematic review characterized the tumor spectrum and clinical features of MEN5.
Study context
- Population
- Patients with germline MAX mutations (MEN5), published studies from PubMed and EMBASE to June 2025
- Participants
- 127 patients45 studies
- Outcome
- Tumor spectrum and clinical features of MEN5
Methodological detailspatient-level data, descriptive analysis
- Patient-level data
- Descriptive analysis
Pooled result
As reported in the abstract
- Median age at first tumor diagnosis
- 29 years (23.0 - 39.0)
- Women
- 37.0%
- Pheochromocytoma (PCC)
- N = 112
- Pituitary adenoma
- N = 11
- Paraganglioma
- N = 10
- Median age at diagnosis: PCC, pituitary adenoma, paraganglioma
- 31.0, 33.0, and 43.0 years
- PCCs unilateral
- 38.3%
- PCCs metastatic
- 27.6%
Original publication
Multiple Endocrine Neoplasia Type 5 due to Germline MAX Mutations: A Systematic Review of Tumor Spectrum and Clinical Features. Endocr Pract. Published 30 December 2025. doi:10.
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