Multiple Endocrine Neoplasia Type 2B

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C3227

A type of multiple endocrine neoplasia occurring as an isolated congenital presentation or as a distinct autosomal dominant disease. It is characterized by the 100% incidence of medullary thyroid carcinoma and frequent pheochromocytomas; patients seldom exhibit hyperparathyroidism. It is distinguished from MEN 2a by its characteristic physical appearance resulting from numerous neural defects including mucosal neuromas of the eyelids, lips, and tongue. The neural abnormalities also include widespread neurogangliomatosis of the gastrointestinal tract leading to abnormal gut motility. Treatment usually requires total thyroidectomy following evaluation for the presence of pheochromocytomas.

C3227

Multiple endocrine neoplasia caused by mutation of the RET gene. Patients develop medullary thyroid carcinomas and numerous neural defects including neuromas.

Multiple Endocrine Neoplasia, Type III

MEN Type 2B

MEN 2B

MEN Type IIB

Multiple Endocrine Adenomatosis Type IIB

Multiple Endocrine Neoplasia Type IIB

Multiple Endocrine Neoplasia Type III

MEN IIB

Multiple Endocrine Neoplasia Type 2B

Multiple_Endocrine_Neoplasia_Type_III

Undetermined

Multiple Endocrine Neoplasia Type 2B

C3227

Multiple Endocrine Neoplasia Type 2B

Neoplastic Process

C0025269

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C123329