๐ ็ธฝ็ฎ้ ๏ฝ ๐ ่ฑๆๅๆ๏ผๆฌ็ฏ๏ผ ๏ฝ ๐ ๅฎๆด็ฟป่ญฏ ๏ฝ โญ ็ฒพ่ฏ็ญ่จ
Mucosal neuroma
Mucosal neuroma
Clinical features Mucosal neuromas are always multiple and have predilection for the mouth.1โ3 They may present as part of the multiple endocrine neoplasia syndrome type IIB (MEN 2B, Sipple; RET at 10q11.21), an autosomal dominant disease characterized by a marfanoid body habitus, dysmorphic facies, medullary carcinoma of thyroid and pheochromocytoma.1โ6 Laryngeal lesions may rarely also present in MEN2B.7โ9 Rare examples may present in the mouth and even the larynx in patients without MEN 2B.10โ12
Pathogenesis and histologic features SOS1 frameshift mutations cause pure mucosal neuroma syndrome unassociated with MEN 2B.13
Histologically, lesions are poorly circumscribed and consist of hyperplastic nerves in a haphazard and disorganized arrangement. An incomplete capsule surrounded by a layer of EMA-positive perineural cells is sometimes present.