Muir-Torre 症候群の1例  [in Japanese] A Case Report of Muir-Torre Syndrome  [in Japanese]

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Muir-Torre症候群は, 脂腺腫瘍あるいはケラトアカントーマと内臓悪性腫瘍を併発するまれな遺伝性疾患である. 今回我々は, 3回の大腸癌切除と1回の胃癌切除の後, 55歳時に顔面のケラトアカントーマおよび前胸部に長径1cmの脂腺癌 (Sebaceous carcinoma) が併せて発生し, 本症候群と診断された症例を経験した. 消化器癌の治療経過は, 37歳時 : 回盲部, 2型, 組織学的深達度ss/右結腸切除施行, 42歳 : 横行結腸, 1型, sm/横行結腸部分切除, 52歳 : 直腸Ra, IIa+IIc型, sm/低位前方切除, 53歳 : 胃幽門前庭部, IIa+IIc, m/ハンドアシスト法腹腔鏡補助下幽門側胃切除術, である. 病理組織診にて, 大腸・胃ともに高分化腺癌でリンパ節転移を認めなかった. 家族歴では祖父に大腸癌, 父に肺癌, 叔父に胃癌を認めた. 58歳時点で無再発生存中である. 本症候群の本邦報告例を中心に集計しその特徴を考察した.

Muir-Torre Syndrome is defined as a rare hereditary disease that is combined with multiple sebaceous tumors and internal malignancies. In a 55-years-old man with facial keratoacanthoma, a left breast tumor of 1cm in diameter was extirpated which revealed sebaceous carcinoma histologically. In the past, he had been operated on four times because of gastrointestinal malignancies : At 37 y.o. : cecal cancer, Type 2, T2N0M0/right colectomy, 42 y.o. : transverse colon ca., Type 1, T1N0M0/partial resection, 52 y.o. : Rectal (Ra) ca., Type IIa+IIc T1N0M0/low anterior resection and at 53 y.o. : gastric cancer Type IIa+IIc, T1N0M0/hand-assisted laparoscopic distal gastrectomy. All tumors were diagnosed as well differentiated adenocarcinoma without lymph node metastasis. His family has a positive family history of internal malignancies : colorectal in his grandfather, lung in his father and stomach in his uncle.


  • The Japanese journal of proctology  

    The Japanese journal of proctology 60(3), 156-160, 2007-03-01 

    The Japan Society of Coloproctology

References:  22

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