下垂体腺腫，ラトケ囊胞を合併したCarney complexの1例

A Case of Carney Complex Complicated with Pituitary Adenoma and Rathke Cleft Cyst

Ⅰ．は じ め に

　Carney complex（CNC）は皮膚の色素沈着異常，粘液腫，内分泌腫瘍や機能亢進，神経鞘腫の合併を特徴とし，常染色体優性遺伝を示す稀な疾患である．脳神経外科領域では10％程度の発症率を持ち，若年者において巨人症を呈する可能性を持つ下垂体腫瘍や，同じく10％程度の発症率を示す神経鞘腫が治療対象となる．しかし内科，皮膚科領域を除き本邦での脳神経外科領域での治療に関してはこれまで報告がなく，このため脳神経外科医の本疾患に対する認識も十分とはいえない．今回われわれは，12歳女児において血清成長ホルモン（s-GH）高値を示し巨人症様症状を呈した稀なCNCの1治療例を経験し，同症例において下垂体腺腫とラトケ囊胞の合併を認めた．総合的CNCに関する文献的考察を踏まえ報告する．

　Carney complex is a rare autosomal-dominant, familial tumor syndrome first described in the mid 80's. This syndrome is multiple neoplasia syndrome featuring cardiac, endocrine, cutaneous, and neural tumor, in addition to a variety of pigmented lesions of the skin and mucosa. We report the case of a 12-year-old female patient with Carney complex who manifested a high value of serum growth hormone (s-GH), cutaneous angiomyxomas and labial pigmented lesions. Magnetic resonance imaging (MRI) revealed a cystic pituitary tumor. We carried out removal of the pituitary tumor via the transsphenoidal approach. In addition to the pituitary adenoma, pathological examination revealed the presence of a Rathke cleft cyst. So far, approximately 500 cases of this disorder have been described, but there have been no cases similar to our case described here. Therefore, the present case seems to be the first case of Carney Complex complicated with pituitary adenoma and Rathke cleft cyst.