1993
DOI: 10.1200/jco.1993.11.2.262
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Second malignant neoplasms in children treated for rhabdomyosarcoma. Intergroup Rhabdomyosarcoma Study Committee.

Abstract: The results of this study suggest that genetic abnormalities play a prominent role in the development of an SMN after therapy for a primary RMS. Chemotherapy with an alkylating agent and radiotherapy play significant roles in the development of an SMN compared with patients who received only one of these therapeutic modalities.

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Cited by 186 publications
(103 citation statements)
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“…This has considerable implications for the clinical management of patients in Li-Fraumeni families, particularly in screening for cancers and for treatment. While the frequency of radiationinduced tumours is still not known in germline mutant TP53 carriers, there is evidence that second malignant neoplasms occur at a relatively high frequency in the radiation zone (Strong and Williams, 1987;Heyn et al, 1993), although more extensive studies must be carried out to confirm this. The relative contributions of genetic background and radiation remain to be evaluated in a careful epidemiological study.…”
Section: Functional Studies Of Li-fraumeni Cellsmentioning
confidence: 99%
See 1 more Smart Citation
“…This has considerable implications for the clinical management of patients in Li-Fraumeni families, particularly in screening for cancers and for treatment. While the frequency of radiationinduced tumours is still not known in germline mutant TP53 carriers, there is evidence that second malignant neoplasms occur at a relatively high frequency in the radiation zone (Strong and Williams, 1987;Heyn et al, 1993), although more extensive studies must be carried out to confirm this. The relative contributions of genetic background and radiation remain to be evaluated in a careful epidemiological study.…”
Section: Functional Studies Of Li-fraumeni Cellsmentioning
confidence: 99%
“…This will probably have more implications for treatment of tumours than in prevention, but any procedure reducing mortality in Li-Fraumeni syndrome would be gratefully accepted by the families. There is a general assumption that tumours in carriers of germline TP53 mutations may be relatively radiation resistant, although hard data are not available and there appears to be a high risk of second malignancy in the radiation field (Strong and Williams, 1987;Heyn et al, 1993). However, more extensive longitudinal studies on germline TP53 mutation carriers are necessary to assess the effectiveness of current treatment regimens.…”
Section: Clinical Aspects Of Li-fraumeni Familiesmentioning
confidence: 99%
“…A role for chem otherapy in treatment is yet to be established, and som e agents may increase the incidence of these rare tum ours. 21,91 …”
Section: Onclusionsmentioning
confidence: 99%
“…An increased risk of STS has been found in survivors of various types of childhood cancers, including retinoblastoma, leukaemia, Wilms' tumour, and Hodgkin's lymphoma (Breslow et al, 1995;Beaty et al, 1995;Moll et al, 1997;Neglia et al, 2001;Bisogno et al, 2004;Fletcher et al, 2004;Kleinerman et al, 2005). Also survivors of childhood STS, osteosarcoma, and Ewing sarcoma have been shown to be at increased risk of multiple primary malignancies, including bone sarcomas and STS (Heyn et al, 1993;Pratt et al, 1997;Neglia et al, 2001;Paulussen et al, 2001;Bacci et al, 2005;Cohen et al, 2005;Bassal et al, 2006). Development of multiple primary malignancies may also represent a side effect of chemotherapy or radiotherapy, which yield an increased risk of secondary malignancies, including STS (Brady et al, 1992;Kony et al, 1997;Neglia et al, 2001;Sheppard and Libshitz, 2001;Cormier and Pollock, 2004;Kirova et al, 2005;Thijssens et al, 2005;Virtanen et al, 2006).…”
mentioning
confidence: 99%