Jass J R, Williams C B, Bussey H J, Morson B C
St Mark's Hospital, London, UK.
Histopathology. 1988 Dec;13(6):619-30. doi: 10.1111/j.1365-2559.1988.tb02093.x.
Clinical and pathological findings in 87 patients with juvenile polyposis have been reviewed; 1032 polyps were available from 80 of these patients; 840 were typical spherical juvenile polyps whereas 169 differed in being multilobulated or showing a villous configuration; 79 (46.7%) of the latter contained foci of epithelial dysplasia whereas only 76 (9.0%) of the typical juvenile polyps were dysplastic. The series also included 21 adenomas and two hyperplastic (metaplastic) polyps. The demonstration of dysplasia provides a histogenetic mechanism for the evolution of colorectal cancer from hamartomatous polyps; 18 juvenile polyposis patients have developed colorectal cancer at a mean age of 34 years (range 15-59). The clinical outcome was generally poor. No clinical or pathological distinction could be made between polyposis patients with and without colorectal cancer. Thus, the development of cancer in juvenile polyposis appears to be a random event. A working definition of juvenile polyposis is provided: (1) more than five juvenile polyps of the colorectum; and/or (2) juvenile polyps throughout the gastrointestinal tract; and/or (3) any number of juvenile polyps with a family history of juvenile polyposis. It is suggested that the condition should be treated as seriously as familial adenomatous polyposis except that regular colonoscopic surveillance may obviate the need for prophylactic colectomy.
对87例幼年性息肉病患者的临床和病理结果进行了回顾；其中80例患者有1032枚息肉；840枚为典型的球形幼年性息肉，而169枚在形态上为分叶状或呈绒毛状；后者中有79枚（46.7%）含有上皮发育异常灶，而典型的幼年性息肉中只有76枚（9.0%）有发育异常。该系列还包括21枚腺瘤和2枚增生性（化生）息肉。发育异常的发现为结直肠癌从错构瘤性息肉演变提供了组织发生学机制；18例幼年性息肉病患者发生了结直肠癌，平均年龄34岁（范围15 - 59岁）。临床结局通常较差。有和没有结直肠癌的息肉病患者之间在临床或病理上没有区别。因此，幼年性息肉病中癌症的发生似乎是一个随机事件。提供了幼年性息肉病的实用定义：（1）结肠直肠有超过5枚幼年性息肉；和/或（2）全胃肠道有幼年性息肉；和/或（3）有幼年性息肉病家族史的任意数量的幼年性息肉。建议应像对待家族性腺瘤性息肉病一样认真对待该疾病，只是定期结肠镜监测可能无需进行预防性结肠切除术。
