multiple endocrine neoplasia
Hereditary syndromes characterized by tumors affecting multiple endocrine glands simultaneously.
Full Definition
Multiple endocrine neoplasia (MEN) syndromes are autosomal dominant genetic disorders that predispose individuals to developing neuroendocrine tumors in multiple glands. MEN1 syndrome involves the parathyroid glands, pancreatic islets, and anterior pituitary, caused by mutations in the MEN1 gene. MEN2 syndrome affects the thyroid (medullary carcinoma), adrenal medulla (pheochromocytoma), and parathyroid glands, resulting from RET proto-oncogene mutations. These syndromes require lifelong surveillance and coordinated management by multidisciplinary teams. Genetic testing and family screening are essential components of care for affected individuals and their relatives.
Usage
Usage note: Often abbreviated as MEN1 or MEN2 based on the specific subtype.
In Context
- "Genetic testing confirmed MEN1 syndrome with a pathogenic mutation in the menin gene." — Genetics consultation report
- "The patient's family history of medullary thyroid carcinoma raised suspicion for MEN2 syndrome." — Clinical assessment