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Mucosal neuromas are benign tumors of nerve tissue that are a characteristic feature of multiple endocrine neoplasia type 2B (also known as MEN3), a hereditary syndrome associated with the development of medullary thyroid cancer.
MEN syndromes comprise 3 genetically distinct familial diseases involving adenomatous hyperplasia and malignant tumors in several endocrine glands.
The genetic defect in these disorders involves the RET proto-oncogene on chromosome 10.
MEN 2B involves primarily medullary thyroid carcinoma, pheochromocytoma, multiple mucosal and intestinal neuromas, and marfanoid habitus
The prevalence of all MEN2 worldwide is 1 in 35,000, while in the United States, it is 1 in 30,000 to 50,000.
The epidemiology of MEN2B is unknown.
The prevalence of MEN2B is estimated to be between 1 in 600,000 to 1 in 4 million.
Multiple endocrine neoplasia (MEN) syndromes are inherited disorders that affect the endocrine system.
Mucosal neuroma is a typical phenotype of MEN 2B, one that develops mostly at birth or at around one to two years. Therefore, early detection of mucosal neuroma is a crucial part of a good prognosis
Mucosal Neuromas | NEJM. (2022). Retrieved 21 November 2022, from https://www.nejm.org/doi/full/10.1056/NEJMicm1815549
https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07735-y