Järvinen H, Franssila K O
Gut. 1984 Jul;25(7):792-800. doi: 10.1136/gut.25.7.792.
Six patients from one family and one solitary patient with juvenile polyposis coli are described. The histological changes in colonic polyps formed a spectrum from juvenile polyps, through focal to extensive adenomatous change, to adenocarcinomas. One patient aged 49 years had an adenocarcinoma of the colon and in another, aged 33, with rectal polyps and metastatic cancer this was suspected although the primary tumour was not located. Two additional patients, aged 19 and 41 years, had severe adenomatous dysplasia in a juvenile polyp. Four patients also had gastroduodenal polyps. The present findings clearly contradict the previous view that juvenile polyposis coli is not premalignant and only rarely needs surgical treatment. As other recent reports also describe frequent occurrence of neoplastic changes in juvenile polyps, colectomy, and ileorectostomy at the age of about 20 years is recommended as the treatment of choice for juvenile polyposis coli, as in patients with familial adenomatosis coli. Follow up should ideally include gastroduodenoscopy and inspection of the rectal remnant at regular intervals.
本文描述了来自一个家族的6例患者以及1例散发性幼年型息肉病患者。结肠息肉的组织学变化呈现出一个范围，从幼年型息肉，经过局灶性到广泛性腺瘤样改变，直至腺癌。一名49岁的患者患有结肠癌，另一名33岁的患者有直肠息肉和转移性癌，尽管原发肿瘤未找到，但怀疑患有此病。另外两名患者，年龄分别为19岁和41岁，在幼年型息肉中存在严重的腺瘤样发育异常。4名患者还患有胃十二指肠息肉。目前的研究结果明显与之前的观点相矛盾，即幼年型息肉病不是癌前病变，且很少需要手术治疗。正如其他近期报告也描述了幼年型息肉中肿瘤性变化的频繁发生一样，对于幼年型息肉病，建议像家族性腺瘤性息肉病患者一样，在大约20岁时进行结肠切除术和回肠直肠吻合术作为首选治疗方法。理想的随访应定期包括胃十二指肠镜检查和直肠残端检查。
