小児の Multiple Endocrine Neoplasia,Type 3 の1例

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  • Medullary Thyroid Carcinoma, Multiple Mucosal Neuroma and Marfanoid Habitus in a boy aged 6 years : Syndrome MEN, Type III
  • ショウニ ノ Multiple Endocrine Neoplasia Typ

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MEN, type III is an extremely rare syndrome. Only six cases have been reported in Japan. Recently we experienced a case with medullary thyroid carcinoma, multiple mucosal neuroma and Marfanoid habitus in childhood, which is the youngest case in Japanese literatures. A 6-year-old boy was admitted with a complaint of left cervical tumors. "Bumpy" lips, Marfanoid habitus and whitish papillary lesions on the tongue tip which was histologically proved plexiform neuroma were characteristic. Megacolon, polyps of colon, diverticulosis coli and medullated corneal nerves were also detected. Under the diagnosis of thyroid cancer, resection of the left lobe of the thyroid with bilateral modified radical neck dissection was carried out. Postoperative calcitonin level remained high (5,674 pg/ml, indicating incomplete removal of the metastatic medullary thyroid carcinoma in the anterior mediastinum. External radiotherapy by 60^Co was performed postoperatively. A series of endocrinological examination failed to find a pheochromocytoma. A careful study of the family history gave a negative result.

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