Zink P M, Tatagiba M, Samii M, Brandis A, Dankoweit-Timpe E
Neurochirurgische Klinik, Landeshauptstadt, Hannover.
Nervenarzt. 1992 Aug;63(8):506-9.
Turcot syndrome denotes the association between either familial polyposis coli (FPC) or sporadic non-familial polyposis coli, and primary neuroepithelial tumors of the central nervous system (CNS). The clinical and morphological intestinal features in both FPC and Turcot syndrome are the same: the histological picture shows typical (tubular) adenomas, often with adenocarcinomatous transformation. As associated CNS tumors astrocytomas, glioblastomas, spongioblastomas, and medulloblastomas are reported. As a modification of ITOH's classification of 1985 we distinguish between four groups: patients with fewer than 100 polyps (group I); patients with fewer than 10 polyps (group II); patients with FPC or sporadic non-familial polyposis (group III); patients with either CNS tumors or FPC with a direct relative with Turcot syndrome and/or FPC (group IV). In patients belonging to the fourth group the diagnosis of Turcot syndrome should not be made. We report on two patients belonging to group II and group IV respectively, and depict the clinical features as well as the heterogenic appearance of Turcot syndrome as outlined in the literature.
Turcot综合征指家族性腺瘤性息肉病（FPC）或散发性非家族性腺瘤性息肉病与中枢神经系统（CNS）原发性神经上皮肿瘤之间的关联。FPC和Turcot综合征的临床及形态学肠道特征相同：组织学表现为典型的（管状）腺瘤，常伴有腺癌转化。报道的相关CNS肿瘤有星形细胞瘤、胶质母细胞瘤、成胶质细胞瘤和髓母细胞瘤。作为对1985年ITOH分类的修订，我们将其分为四组：息肉少于100个的患者（I组）；息肉少于10个的患者（II组）；患有FPC或散发性非家族性腺瘤性息肉病的患者（III组）；患有CNS肿瘤或FPC且有Turcot综合征和/或FPC直系亲属的患者（IV组）。对于属于第四组的患者，不应诊断为Turcot综合征。我们分别报告了II组和IV组的两名患者，并描述了Turcot综合征的临床特征以及文献中概述的异质性表现。
