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Published on: August 25, 2023
Multiple endocrine neoplasia type 1
1NW Thompson Professor of Surgery, University of Michigan, Ann Arbor, Michigan, USA. gerardd@umich.edu
Abstract:
Multiple endocrine neoplasia-1 (MEN-1) is an autosomal dominant inherited syndrome that occurs due to inactivating mutations of the MEN1 gene locus, coding for a tumor-suppressor protein, menin. The components of MEN-1 are hyperparathyroidism due to multiple parathyroid adenomas, pancreatic neuroendocrine tumors, and pituitary adenomas, in addition to some less common neoplastic manifestations. Care of people with MEN-1 requires knowledge of the problems that may arise, and the best approaches to detect and care for the manifestations of this incurable, but manageable, disease.
Insights
Multiple endocrine neoplasia-1 (MEN-1) is an inherited syndrome caused by MEN1 gene mutations. Management focuses on detecting and caring for associated tumors like parathyroid, pancreatic, and pituitary adenomas.
Area of Science:
- Endocrinology
- Genetics
- Oncology
Background:
- Multiple endocrine neoplasia-1 (MEN-1) is an autosomal dominant inherited syndrome.
- It results from inactivating mutations in the MEN1 gene, which encodes the tumor-suppressor protein menin.
- MEN-1 is characterized by specific neoplastic manifestations.
Purpose of the Study:
- To outline the key features and manifestations of MEN-1.
- To emphasize the importance of understanding and managing this condition.
- To provide guidance on detecting and caring for MEN-1 associated tumors.
Main Methods:
- Review of the genetic basis of MEN-1, focusing on MEN1 gene mutations.
- Description of the common and less common neoplastic manifestations.
- Discussion of clinical management strategies for MEN-1 patients.
Main Results:
- MEN-1 commonly involves hyperparathyroidism (parathyroid adenomas), pancreatic neuroendocrine tumors, and pituitary adenomas.
- Less frequent neoplastic conditions can also occur in MEN-1 patients.
- Early detection and proactive management are crucial for patient care.
Conclusions:
- MEN-1 is a complex inherited endocrine neoplasia syndrome.
- While incurable, MEN-1 is manageable with comprehensive care.
- Knowledge of potential complications and management approaches is essential for healthcare providers.
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