Itoh H, Hirata K, Ohsato K
Department of Surgery I, University of Occupational and Environmental Health, Kitakyushu, Japan.
Int J Colorectal Dis. 1993 Jul;8(2):87-94. doi: 10.1007/BF00299334.
We investigated clinical manifestations in 124 patients with a possible Turcot's syndrome whose data were taken from documented cases. The cases were subclassified mainly on the bases of the type of familial occurrence and listed in five Tables. We searched for differences in colonic manifestation, histologic type of glioma, mode of inheritance, frequency of parental consanguinity, skin lesions and other accompanying lesions among these five groups. The differences of these clinical findings suggested that glioma-polyposis syndrome should be classified as follows; (1) cases of Turcot's syndrome who had characteristic colonic and brain manifestations, (2) cases of FAP associated with glioma, (3) suspicious cases of glioma-polyposis, and (4) cases other than glioma-polyposis syndrome.
我们调查了124例可能患有图尔科特综合征患者的临床表现，其数据取自已记录的病例。这些病例主要根据家族发病类型进行亚分类，并列于五个表格中。我们在这五组患者中寻找结肠表现、胶质瘤组织学类型、遗传方式、父母近亲结婚频率、皮肤病变及其他伴随病变方面的差异。这些临床发现的差异表明，胶质瘤-息肉病综合征应分类如下：（1）具有典型结肠和脑部表现的图尔科特综合征病例；（2）与胶质瘤相关的家族性腺瘤性息肉病（FAP）病例；（3）胶质瘤-息肉病可疑病例；（4）胶质瘤-息肉病综合征以外的病例。
