Patterns of cancer in the families of children with soft tissue sarcoma.

2.50
Hdl Handle:
http://hdl.handle.net/10541/99617
Title:
Patterns of cancer in the families of children with soft tissue sarcoma.
Authors:
Hartley, Ann L; Birch, Jillian M; Blair, Val; Kelsey, Anna M; Harris, Martin; Jones, P H
Abstract:
BACKGROUND: Childhood soft tissue sarcomas are known to occur in a number of genetic syndromes. This study assesses the proportion of soft tissue sarcoma diagnosed in childhood associated with genetic predisposition to cancer. METHODS: Information on the occurrence of neoplastic disease was collected for 151 of 179 families of a population-based series of children with soft tissue sarcoma. RESULTS: Considering the index child as the proband, 5 of the 151 families manifested the classic Li-Fraumeni cancer family syndrome according to standard criteria and a further 10 families showed features consistent with the syndrome. One proband had double primary syndrome cancers. One other family had a sibling pair of childhood cancers, seven families had cancer which had occurred in childhood in other relatives, and three families had adult-onset sarcomas in more distant relatives. In another 16 families, one parent or the other had developed a possible syndrome cancer, or had developed cancer when younger than 60 years of age. Two families showed striking clusters of stomach cancer. Five case children were thought to have been affected with neurofibromatosis. CONCLUSIONS: Genetic predisposition to cancer was thought to be present in 7% to 33% of families interviewed.
Affiliation:
Department of Epidemiology and Social Oncology, Christie Hospital NHS Trust, Manchester, UK.
Citation:
Patterns of cancer in the families of children with soft tissue sarcoma. 1993, 72 (3):923-30 Cancer
Journal:
Cancer
Issue Date:
1-Aug-1993
URI:
http://hdl.handle.net/10541/99617
DOI:
10.1002/1097-0142(19930801)72:3<923::AID-CNCR2820720343>3.0.CO;2-Y
PubMed ID:
8334646
Type:
Article
Language:
en
ISSN:
0008-543X
Appears in Collections:
All Christie Publications

Full metadata record

DC FieldValue Language
dc.contributor.authorHartley, Ann Len
dc.contributor.authorBirch, Jillian Men
dc.contributor.authorBlair, Valen
dc.contributor.authorKelsey, Anna Men
dc.contributor.authorHarris, Martinen
dc.contributor.authorJones, P Hen
dc.date.accessioned2010-05-21T14:34:41Z-
dc.date.available2010-05-21T14:34:41Z-
dc.date.issued1993-08-01-
dc.identifier.citationPatterns of cancer in the families of children with soft tissue sarcoma. 1993, 72 (3):923-30 Canceren
dc.identifier.issn0008-543X-
dc.identifier.pmid8334646-
dc.identifier.doi10.1002/1097-0142(19930801)72:3<923::AID-CNCR2820720343>3.0.CO;2-Y-
dc.identifier.urihttp://hdl.handle.net/10541/99617-
dc.description.abstractBACKGROUND: Childhood soft tissue sarcomas are known to occur in a number of genetic syndromes. This study assesses the proportion of soft tissue sarcoma diagnosed in childhood associated with genetic predisposition to cancer. METHODS: Information on the occurrence of neoplastic disease was collected for 151 of 179 families of a population-based series of children with soft tissue sarcoma. RESULTS: Considering the index child as the proband, 5 of the 151 families manifested the classic Li-Fraumeni cancer family syndrome according to standard criteria and a further 10 families showed features consistent with the syndrome. One proband had double primary syndrome cancers. One other family had a sibling pair of childhood cancers, seven families had cancer which had occurred in childhood in other relatives, and three families had adult-onset sarcomas in more distant relatives. In another 16 families, one parent or the other had developed a possible syndrome cancer, or had developed cancer when younger than 60 years of age. Two families showed striking clusters of stomach cancer. Five case children were thought to have been affected with neurofibromatosis. CONCLUSIONS: Genetic predisposition to cancer was thought to be present in 7% to 33% of families interviewed.en
dc.language.isoenen
dc.subjectSoft Tissue Canceren
dc.subject.meshChild-
dc.subject.meshChild, Preschool-
dc.subject.meshFemale-
dc.subject.meshHumans-
dc.subject.meshLi-Fraumeni Syndrome-
dc.subject.meshMale-
dc.subject.meshPedigree-
dc.subject.meshRhabdomyosarcoma-
dc.subject.meshSarcoma-
dc.subject.meshSoft Tissue Neoplasms-
dc.titlePatterns of cancer in the families of children with soft tissue sarcoma.en
dc.typeArticleen
dc.contributor.departmentDepartment of Epidemiology and Social Oncology, Christie Hospital NHS Trust, Manchester, UK.en
dc.identifier.journalCanceren

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