[Turcot syndrome illustrated by two clinical cases].
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.
Zink
P M
PM
Neurochirurgische Klinik, Landeshauptstadt, Hannover.
Tatagiba
M
M
Samii
M
M
Brandis
A
A
Dankoweit-Timpe
E
E
D011125:Adenomatous Polyposis Coli / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D000328:Adult; D001921:Brain / Q000473:pathology; D016543:Central Nervous System Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D005910:Glioma / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D006801:Humans; D007413:Intestinal Mucosa / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009386:Neoplastic Syndromes, Hereditary / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D009447:Neuroblastoma / Q000175:diagnosis / Q000235:genetics / Q000473:pathology
Die Problematik des Turcot-Syndroms, aufgezeigt an zwei klinischen Fallbeispielen.