多発性内分泌腫瘍症 (MEN2B) に伴う口腔粘膜神経腫の1例

抄録

Multiple endocrine neoplasia (MEN) include three genetically distinct disorders: MEN 1, MEN 2 A, and MEN 2 B. These three syndromes are inherited as autosomal dominant traits, although each may also occur sporadically. MEN 2 B is characterized by medullary thyroid carcinoma, pheochromocytomas, mucosal neuromas, ganglioneuromas of the intestinal tract, and Marfan's syndrome. The case of a 27-year-old man with MEN 2 B is reported. The lips were diffusely enlarged, with distinct projections at the central part of the upper and lower lips caused by neuromatous enlargement. The chief involvement of the tongue affected the dorsum and appeared as multiple nodules of varying size. Biopsies of some nodules of the tongue were obtained. Histological and immunohistochemical studies (HE, S-100 protein and NSE) showed hyperplasia and hypertrophy of nerves, suggesting neuroma. The oral aspects of MEN 2 B are important since they are the initial manifestations of this disorder, preceding thyroid carcinoma by as much as 10 to 20 years. Failure to recognize the syndrome may have unfortunate consequences for the patient, since proper treatment of the thyroid tumor that may arise in childhood will not be provided. Therefore, it is vary important for dentists and physicians who treat oral diseases to carefully examine the oral cavity.