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MEN2B-associated multiple mucosal neuroma

Friday 17 February 2012

mucosal neuroma syndrome; myelinic neuroma

Multiple endocrine neoplasia type 2B (MEN 2B) is a rare disease caused by germline mutations in the RET proto-oncogene and is transmitted in an autosomal dominant fashion.

Multiple endocrine neoplasia type 2B (MEN 2B) is characterized by medullary thyroid carcinoma, pheochromocytoma and mucosal neuroma developing in the tongue, lip, intestinal tract, palate etc.

Among these neoplasias, mucosal neuroma generally develops from early childhood. Therefore, early detection and proper treatment can minimize the disease course.

Histologic findings of his lips and tongue show well-defined nerve bundles.

Open references

- Multiple Endocrine Neoplasia Type 2B: Early Diagnosis by Multiple Mucosal Neuroma and Its DNA Analysis. Lee MJ, Chung KH, Park JS, Chung H, Jang HC, Kim JW. Ann Dermatol. 2010 Nov;22(4):452-5. PMID: 21165219 (Free)