Pietroiusti A, Caprilli R, Giuliano M, Serrano S
Cancer. 1986 Jun 15;57(12):2438-40. doi: 10.1002/1097-0142(19860615)57:12<2438::aid-cncr2820571231>3.0.co;2-f.
A large family with a high prevalence of colorectal cancer, presenting the major features of hereditary site-specific colon cancer (i.e., autosomic dominant model of heredity, absence of associated adenomatous polyposis, prevalence of right-sided colonic lesions, and young age of the patients) is described. The lack of association with solitary adenomatous polyps, the higher survival rate of patients with left-sided colon cancer, and the absence of extracolonic tumors were peculiar characteristics of this family. These data, in agreement with other reports, appear to suggest that the inherited colon cancer syndromes are not a single entity, but a group of related entities linked by their familial occurrence.
描述了一个结直肠癌高患病率的大家族,其呈现出遗传性位点特异性结肠癌的主要特征(即常染色体显性遗传模式、无相关腺瘤性息肉、右侧结肠病变患病率高以及患者发病年龄较轻)。该家族的独特特征包括与孤立性腺瘤性息肉无关、左侧结肠癌患者生存率较高以及无结肠外肿瘤。与其他报告一致,这些数据似乎表明遗传性结肠癌综合征并非单一实体,而是一组因家族性发病而相关的实体。