Lynch H T, Smyrk T C, Lynch P M, Lanspa S J, Boman B M, Ens J, Lynch J F, Strayhorn P, Carmody T, Cristofaro G
Department of Preventive Medicine/Public Health, M.D. Anderson Cancer Center, Houston, Texas.
Cancer. 1989 Nov 15;64(10):2178-83. doi: 10.1002/1097-0142(19891115)64:10<2178::aid-cncr2820641033>3.0.co;2-0.
Adenocarcinoma of the small bowel is rare and accounts for about 1% of all gastrointestinal tract cancer. This disorder has been identified in association with Crohn's disease, celiac disease, Peutz-Jegher's syndrome, and familial adenomatous polyposis. We report adenocarcinoma of the small bowel in nine patients from eight Lynch syndrome II extended pedigrees. Each affected patient was in the direct genetic lineage or manifested multiple primary cancers (stomach, colon, endometrium, and ovary) consonant with the tumor spectrum of Lynch syndrome II. The average age of onset for small bowel cancer was 47 years (range 31 to 56 years), versus the general population peak occurrence after the sixth decade. We conclude that small bowel cancer may be an integral component of the tumor spectrum of Lynch syndrome II.
小肠腺癌较为罕见，约占所有胃肠道癌症的1%。已发现该疾病与克罗恩病、乳糜泻、黑斑息肉综合征和家族性腺瘤性息肉病相关。我们报告了来自8个林奇综合征II型扩展家系的9例小肠腺癌患者。每例受影响患者均处于直接遗传谱系中，或表现出与林奇综合征II型肿瘤谱相符的多种原发性癌症（胃癌、结肠癌、子宫内膜癌和卵巢癌）。小肠癌的平均发病年龄为47岁（范围31至56岁），而普通人群的发病高峰在60岁以后。我们得出结论，小肠癌可能是林奇综合征II型肿瘤谱的一个组成部分。
