Clinical manifestation

Oral mucosal neuroma: yellow-white, sessile, painless papule of the lips, anterior tongue, and buccal commissures; similar lesions seen on the eyelids, sometimes producing eversion of the lid, and on the sclera; facial skin, especially around the nose, sometimes involved; lesions develop in first decade of life; tall, lanky, marfanoid body type, with a narrow face and muscle wasting; adrenal and thyroid tumors present after puberty; associated with adrenal phe-ochromocytoma, medullary thyroid carcinoma, diffuse alimentary tract ganglioneu-romatosis, and multiple, small, submucosal neuroma nodules of the upper aerodiges-tive tract

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