We believe that the description of Turcot's syndrome by Rustgi maintains the confusion that has existed in the literature since the original description of the association between polyposis and central nervous system tumors. We think that there are two distinct conditions: autosomal dominant familial adenomatous polyposis with central nervous system tumors as extracolonic features and autosomal recessive Turcot's syndrome with central nervous system tumors as primary features.
Dupuis, MJM., & Dumoulin, C. (1995). Gastrointestinal Polyposis and Nonpolyposis Syndromes. New England Journal of Medicine, 332(22), 1518. https://doi.org/10.1056/NEJM199506013322215 (Original work published 1995)