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dc.contributor.authorBirch, Jillian M
dc.contributor.authorHartley, Ann L
dc.contributor.authorBlair, Val
dc.contributor.authorKelsey, Anna M
dc.contributor.authorHarris, Martin
dc.contributor.authorTeare, M Dawn
dc.contributor.authorJones, P H
dc.date.accessioned2010-08-16T15:13:44Z
dc.date.available2010-08-16T15:13:44Z
dc.date.issued1990-11-15
dc.identifier.citationCancer in the families of children with soft tissue sarcoma. 1990, 66 (10):2239-48 Canceren
dc.identifier.issn0008-543X
dc.identifier.pmid2224780
dc.identifier.doi10.1002/1097-0142(19901115)66:10<2239::AID-CNCR2820661034>3.0.CO;2-Q
dc.identifier.urihttp://hdl.handle.net/10541/109663
dc.description.abstractThe cancer experience among 754 first-degree relatives (mothers, fathers, and siblings) of a population-based series of 177 children with soft tissue sarcoma is reported. The current study represents an extension of our earlier work in which the authors found an excess of breast cancer in the mothers of 143 of these children. There were 40 cancers among all first-degree relatives, compared with 24.82 expected (relative risk [RR] 1.61, P = 0.006). There was no excess in fathers, but an excess of borderline significance was seen in mothers (RR 1.67, P = 0.0545), and a significant excess in siblings (RR 4.55, P = 0.0002), mainly due to carcinoma of the breast and pediatric tumors. Results of a step forward Cox multivariate analysis identified three variables in the index child which were independently associated with high cancer risk in relatives, as follows: age younger than 24 months at diagnosis; histologic type, embryonal rhabdomyosarcoma or other and unspecified soft tissue sarcoma; and male sex. It was possible, therefore, to identify a subgroup of children whose relatives are at high risk of early onset cancer (RR in this group 10.14). The pattern of cancers is consistent with the Li-Fraumeni syndrome. The authors conclude that a marked proportion of childhood soft tissue sarcoma has a genetic basis.
dc.language.isoenen
dc.subjectCanceren
dc.subjectSoft Tissue Canceren
dc.subject.meshAdolescent
dc.subject.meshAdult
dc.subject.meshChild
dc.subject.meshFamily Health
dc.subject.meshFemale
dc.subject.meshHumans
dc.subject.meshMale
dc.subject.meshMiddle Aged
dc.subject.meshNeoplasms
dc.subject.meshPedigree
dc.subject.meshSarcoma
dc.subject.meshSoft Tissue Neoplasms
dc.titleCancer in the families of children with soft tissue sarcoma.en
dc.typeArticleen
dc.contributor.departmentDepartment of Epidemiology & Social Oncology, Christie Hospital & Holt Radium Institute, Manchester, United Kingdom.en
dc.identifier.journalCanceren
html.description.abstractThe cancer experience among 754 first-degree relatives (mothers, fathers, and siblings) of a population-based series of 177 children with soft tissue sarcoma is reported. The current study represents an extension of our earlier work in which the authors found an excess of breast cancer in the mothers of 143 of these children. There were 40 cancers among all first-degree relatives, compared with 24.82 expected (relative risk [RR] 1.61, P = 0.006). There was no excess in fathers, but an excess of borderline significance was seen in mothers (RR 1.67, P = 0.0545), and a significant excess in siblings (RR 4.55, P = 0.0002), mainly due to carcinoma of the breast and pediatric tumors. Results of a step forward Cox multivariate analysis identified three variables in the index child which were independently associated with high cancer risk in relatives, as follows: age younger than 24 months at diagnosis; histologic type, embryonal rhabdomyosarcoma or other and unspecified soft tissue sarcoma; and male sex. It was possible, therefore, to identify a subgroup of children whose relatives are at high risk of early onset cancer (RR in this group 10.14). The pattern of cancers is consistent with the Li-Fraumeni syndrome. The authors conclude that a marked proportion of childhood soft tissue sarcoma has a genetic basis.


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