The Glioma-Polyposis Syndrome

Baughman, Fred A.; List, Carl F.; Williams, John R.; Muldoon, James; Segarra, Joseph M.; Volkel, James S.

doi:10.1056/nejm196912112812407

Cited by 86 publications

(11 citation statements)

References 3 publications

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“…Later, similar reports were made by Yaffee [28], Camiel [29], and Baughan [30], and these complications were termed the Turcot Syndrome. In another aspect, attention has been focused on the complication of GS with a variety of pathologic changes.…”

Section: Lesion~ Associated With F P C and Gs In Organs Othermentioning

confidence: 72%

Lesions associated with familial polyposis coli: Studies of lesions of the stomach, duodenum, bones, and teeth

Ushio

Sasagawa

Doi

et al. 1976

Gastrointest Radiol

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The entire gastrointestinal tract and bones were examined systematically in 24 patients (10 pedigrees) in whom familial polyposis coli was diagnosed. Polypoid lesions were observed in the stomach in as high as 68.2% of the cases, and in the duodenum in 90%. Abnormalities were noted in the skeleton in 50% and in the mandible in 81.3%. The authors emphasize the possibility that familial polyposis coli is substantially the same entity as Gardner's Syndrome.

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Section: Lesion~ Associated With F P C and Gs In Organs Othermentioning

confidence: 72%

Lesions associated with familial polyposis coli: Studies of lesions of the stomach, duodenum, bones, and teeth

Ushio

Sasagawa

Doi

et al. 1976

Gastrointest Radiol

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“…As concerns the pattern of inheritance of Turcot's syndrome, we believe it is unlikely that a recessive gene is involved in the Type I sibships reported by Turcot et ul.,' Baughman et u1., 6 and Itoh et ul." Statistically, only 25% of siblings should be affected by a recessive trait.…”

Section: Discussionmentioning

confidence: 94%

Turcot's syndrome evidence for autosomal dominant inheritance

Lewis

Ginsberg

Toomey

1983

Cancer

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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.

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“…Smith and Kern (1973) speculated that brain tumour represents one of the phenotypes of a dominant gene capable of producing familial multiple polyposis which is transmitted in the autosomal dominant mode. McKusick (1962) and Baughman et al (1969), however, after a study of the family pedigrees, suggested that the mode of inheritance was autosomal recessive. As only a small number of familial cases of Turcot's syndrome have been found, it has been difficult to determine which of the two modes of inheritance is actually responsible for its transmission.…”

Section: Discussionmentioning

confidence: 99%