Turcot's syndrome with intestinal lymphoma in a child: An unusual case of triple tumor

We investigated clinical manifestations in 124 patients with a possible Turcot's syndrome whose data were taken from documented cases. The cases were subclassified mainly on the bases of the type of familial occurrence and listed in five Tables. We searched for differences in colonic manifestation, histologic type of glioma, mode of inheritance, frequency of parental consanguinity, skin lesions and other accompanying lesions among these five groups. The differences of these clinical findings suggested that glioma-polyposis syndrome should be classified as follows; (1) cases of Turcot's syndrome who had characteristic colonic and brain manifestations, (2) cases of FAP associated with glioma, (3) suspicious cases of glioma-polyposis, and (4) cases other than glioma-polyposis syndrome.

show abstract

Turcot's syndrome and familial adenomatous polyposis associated with brain tumor: review of related literature

Itoh

Hirata

Ohsato

1993

Int J Colorect Dis

View full text Add to dashboard Cite

show abstract

Glioblastoma multiforme in four siblings: A cytogenetic and molecular genetic study

Cm¹,

Tuerlings

Wm³

et al. 1995

J Neuro-Oncol

View full text Add to dashboard Cite

The familial occurrence of gliomas, in the absence of well-defined neurological tumor syndromes such as the neurofibromatoses, is uncommon. We present a family of ten children in which the four eldest suffered from gliomas. Three of these siblings had histologically verified glioblastoma multiforme, and one patient also had an intestinal non-Hodgkin's lymphoma, but there were no stigmata or family history of a neurological tumor syndrome. Cytogenetic studies of the proband revealed a normal karyotype. Molecular genetic analysis of the proband's glioblastoma revealed two mutations in the p53 tumor suppressor gene, but these were not present in the germline DNA, mutations were not detected in the MTS1 gene in the tumors or in the germline DNA. These findings suggest that a genetic factor may be responsible for the clustering of glial tumors in this family, but it is unlikely that the genetic alteration is mutation of the p53 gene. The data are discussed in light of the literature on familial brain tumors.

show abstract

An unusual case of Turcot’s syndrome associated with ileal adenocarcinoma, intestinal non-Hodgkin’s lymphoma, and duodenal adenocarcinoma. Review of the classification and genetic basis of Turcot’s syndrome

et al. 2005

View full text Add to dashboard Cite

A 38-year-old man with a history of colonic and small bowel polyposis and glioblastoma was investigated for dyspepsia. Upper GI endoscopy identified an abnormal area in the duodenum, confirmed by histology as high grade non-Hodgkin's B cell MALT lymphoma. Although cases of Turcot's syndrome (TS) (colonic polyposis and primary brain tumour occurring in the same patient) have been previously described, association with haematological malignancy is rare. This is the first report of intestinal lymphoma occurring in an adult with TS.

show abstract

scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.

Contact Info

hi@scite.ai

10624 S. Eastern Ave., Ste. A-614

Henderson, NV 89052, USA

Blog Terms and Conditions API Terms Privacy Policy Contact Cookie Preferences Do Not Sell or Share My Personal Information

Made with 💙 for researchers

Part of the Research Solutions Family.

Turcot's syndrome with intestinal lymphoma in a child: An unusual case of triple tumor

Cited by 6 publications

References 16 publications

Turcot's syndrome and familial adenomatous polyposis associated with brain tumor: review of related literature

Turcot's syndrome and familial adenomatous polyposis associated with brain tumor: review of related literature

Glioblastoma multiforme in four siblings: A cytogenetic and molecular genetic study

An unusual case of Turcot’s syndrome associated with ileal adenocarcinoma, intestinal non-Hodgkin’s lymphoma, and duodenal adenocarcinoma. Review of the classification and genetic basis of Turcot’s syndrome

Contact Info

Product

Resources

About