Turcot's syndrome evidence for autosomal dominant inheritance

A case of Turcot's syndrome (colonic polyposis plus a malignant central nervous system tumor) occurring in a kindred with autosomal dominant colonic polyposis is presented. It is proposed that Turcot's syndrome patients can be classified into Type I where only siblings are affected and Typ...

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Bibliographic Details
Published in:Cancer 1983-02, Vol.51 (3), p.524-528
Main Authors: Lewis, James H., Ginsberg, Allen L., Toomey, Kathleen E.
Format: Article
Language:English
Subjects:
Cerebellar Neoplasms - genetics
Cerebellar Neoplasms - surgery
Child
Colonic Neoplasms - genetics
Colonic Neoplasms - surgery
Genes, Dominant
Humans
Intestinal Polyps - genetics
Intestinal Polyps - surgery
Male
Medulloblastoma - genetics
Medulloblastoma - surgery
Neoplasms, Multiple Primary - genetics
Pedigree
Phenotype
Syndrome
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Summary:A case of Turcot's syndrome (colonic polyposis plus a malignant central nervous system tumor) occurring in a kindred with autosomal dominant colonic polyposis is presented. It is proposed that Turcot's syndrome patients can be classified into Type I where only siblings are affected and Type II where two or more generations have colonic polyposis. A third nonfamilial group cannot be classified into Type I or II based on available information. Evidence is presented suggesting Turcot's syndrome is best considered an additional phenotype of familial polyposis and is most likely inherited in an autosomal dominant manner.
ISSN:0008-543X
1097-0142
DOI:10.1002/1097-0142(19830201)51:3<524::AID-CNCR2820510326>3.0.CO;2-I