Sipple症候群の2症例

Sipple's Syndrome; Report of Two Cases

　はじめに

　Sipple症候群は甲状腺髄様癌（以下MCT），副腎褐色細胞腫まれに上皮小体の腺腫あるいは過形成を合併する疾患で，多内分泌腺腫症（multipleendocrine neoplasia, MEN）のType II_aに分類される比較的稀な疾患である。また常染色体優性遺伝の遺伝形式をとり，家系内発症を多く認める。

　本邦では褐色細胞腫を多く取り扱う，内科，泌尿器科よりの報告が多い。しかし，本疾患は頸部腫瘤を初発症状とすることも少なからずあり，頭頸部領域疾患を取り扱うわれわれも見逃してはならない疾患の1つである。今回，われわれは本疾患2例を経験したので本邦報告例に若干の考察を加えて報告する。

The first case, a 59-year-old male, was admitt-ed to department of Urology because of pheoc-romocytoma. Thyroid tumors were found in both lobes. Enucleation of the tumors and adrenectomy were performed. The tumors were hitologically diagnosed as medullary carcinoma of the thyroid (MCT). So total thyroidectomy was performed after 4 weeks.

The second case, a 47-year-old female, was admitted because of MCT. There was no sym-ptom based on pheocromocytoma. But Abdo-minal MR image revealed left adrenal tumor. Urological examination showed the tumor was pheocromocytoma. So MCT and pheocromocy-toma were removed.