In Endocrine Neoplasia Syndrome

ICD-O:9570/0

Multiple endocrine neoplasia syndrome type 2B is an autosomal dominant condition characterised by the presence of mucosal neuromas together with medullary carcinoma of the thyroid gland and phaeochromocy-toma [147]. Nearly 90% of patients with the condition have point mutations at codon 918 of the RET proto-on-cogene.

Clinically, patients often have a Marfanoid habitus with arachnodactyly and a narrow face. Mucosal neuromas are the most consistent feature of the disease and may be pathognomonic. They tend to form on the lateral margins and dorsum of the tongue and appear as multiple, small, painless nodules. These nodules may be the first indication of the condition. The lips are sometimes enlarged and blubbery. Mucosal neuromas often affect the palpebral conjunctiva and can also involve the sclera.

Microscopy shows a partially encapsulated tangled mass of small nerve fibres, often with a thickened peri-neurium. These nerves lie in a loose fibrous stroma. The nerves usually stain with S-100 and may stain with epithelial membrane antigen indicating perineurial differentiation.

The mucosal neuromas themselves rarely cause clinical problems, but may act as indicators of the more serious aspects of the syndrome.

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