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要旨●家族性大腸腺腫症(FAP)は大腸癌ならびに多様な随伴病変を発症する常染色体優性遺伝性疾患であり,頻度は低いものの,空・回腸癌も随伴病変として知られている.当科の151例のFAPのうち空・回腸癌を発症した4例を提示する.内訳は空腸癌が1例,回腸癌が3例であり,回腸癌のうち2例は人工肛門部に発生した.生涯発症リスク(70歳)は15.7%であった.FAPに対する適切な手術治療とサーベイランスの進歩により,若年での大腸癌による死亡が減少することから,今後は空・回腸癌の発生が増加する可能性があり,症例の蓄積による発生メカニズムの解明とサーベイランスの確立が望まれる.
FAP(familial adenomatous polyposis)is an autosomal dominant inherited disease in which patients develop colorectal cancer and various associated lesions. Among the 151 cases of FAP in our department ; here, we present four cases of jejunal and ileal cancer:one case of jejunal cancer and three cases of ileal cancer, with two of the ileal cancer cases occurring at the colostomy site. Our analysis shows that the lifetime risk(at 70 years of age)of developing jejunal and ileal cancer is 15.7%. However, because appropriate surgical treatment for FAP and advances in surveillance can reduce the number of deaths due to colorectal cancer at a young age, the incidence of jejunal and ileal cancer may increase in the future. Therefore, we hope that a carcinogenic mechanism will be elucidated and that surveillance will be established by the accumulation of cases.
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