MUCOSAL NEUROMA SYNDROME
\mjuːkˈə͡ʊzə͡l njuːɹˈə͡ʊmə sˈɪndɹə͡ʊm], \mjuːkˈəʊzəl njuːɹˈəʊmə sˈɪndɹəʊm], \m_j_uː_k_ˈəʊ_z_əl n_j_uː_ɹ_ˈəʊ_m_ə s_ˈɪ_n_d_ɹ_əʊ_m]\
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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.
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