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. 2003 Apr;88(4):354–357. doi: 10.1136/adc.88.4.354

Update on childhood rhabdomyosarcoma

H McDowell 1
PMCID: PMC1719509  PMID: 12651771

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Figure 1.

Figure 1

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Distribution of RMS by primary site.

Figure 2.

Figure 2

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Diagram of the balanced chromosomal translocations resulting in the fusion genes involving PAX3 and PAX7 and the forkhead gene on chromosome 13.

Figure 3.

Figure 3

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Survival of UKCCSG patients diagnosed 1977–98 by trial entry period. All stages included.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Anderson J., Gordon A., Pritchard-Jones K., Shipley J. Genes, chromosomes, and rhabdomyosarcoma. Genes Chromosomes Cancer. 1999 Dec;26(4):275–285. [PubMed] [Google Scholar]
  2. Baker K. S., Anderson J. R., Link M. P., Grier H. E., Qualman S. J., Maurer H. M., Breneman J. C., Wiener E. S., Crist W. M. Benefit of intensified therapy for patients with local or regional embryonal rhabdomyosarcoma: results from the Intergroup Rhabdomyosarcoma Study IV. J Clin Oncol. 2000 Jun;18(12):2427–2434. doi: 10.1200/JCO.2000.18.12.2427. [DOI] [PubMed] [Google Scholar]
  3. Baldini E. H., Goldberg J., Jenner C., Manola J. B., Demetri G. D., Fletcher C. D., Singer S. Long-term outcomes after function-sparing surgery without radiotherapy for soft tissue sarcoma of the extremities and trunk. J Clin Oncol. 1999 Oct;17(10):3252–3259. doi: 10.1200/JCO.1999.17.10.3252. [DOI] [PubMed] [Google Scholar]
  4. Bargonetti Jill, Manfredi James J. Multiple roles of the tumor suppressor p53. Curr Opin Oncol. 2002 Jan;14(1):86–91. doi: 10.1097/00001622-200201000-00015. [DOI] [PubMed] [Google Scholar]
  5. Barr F. G. Gene fusions involving PAX and FOX family members in alveolar rhabdomyosarcoma. Oncogene. 2001 Sep 10;20(40):5736–5746. doi: 10.1038/sj.onc.1204599. [DOI] [PubMed] [Google Scholar]
  6. Birch J. M., Hartley A. L., Blair V., Kelsey A. M., Harris M., Teare M. D., Jones P. H. Cancer in the families of children with soft tissue sarcoma. Cancer. 1990 Nov 15;66(10):2239–2248. doi: 10.1002/1097-0142(19901115)66:10<2239::aid-cncr2820661034>3.0.co;2-q. [DOI] [PubMed] [Google Scholar]
  7. Breneman J. C., Wiener E. S. Issues in the local control of rhabdomyosarcoma. Med Pediatr Oncol. 2000 Aug;35(2):104–109. doi: 10.1002/1096-911x(200008)35:2<104::aid-mpo3>3.0.co;2-r. [DOI] [PubMed] [Google Scholar]
  8. Carli M., Colombatti R., Oberlin O., Stevens M., Masiero L., Frascella E., Koscielniak E., Treuner J., Pinkerton C. R. High-dose melphalan with autologous stem-cell rescue in metastatic rhabdomyosarcoma. J Clin Oncol. 1999 Sep;17(9):2796–2803. doi: 10.1200/JCO.1999.17.9.2796. [DOI] [PubMed] [Google Scholar]
  9. Crist W. M., Anderson J. R., Meza J. L., Fryer C., Raney R. B., Ruymann F. B., Breneman J., Qualman S. J., Wiener E., Wharam M. Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. J Clin Oncol. 2001 Jun 15;19(12):3091–3102. doi: 10.1200/JCO.2001.19.12.3091. [DOI] [PubMed] [Google Scholar]
  10. Gaiger A. M., Soule E. H., Newton W. A., Jr Pathology of rhabdomyosarcoma: experience of the Intergroup Rhabdomyosarcoma Study, 1972-78. Natl Cancer Inst Monogr. 1981 Apr;(56):19–27. [PubMed] [Google Scholar]
  11. Gordon T., McManus A., Anderson J., Min T., Swansbury J., Pritchard-Jones K., Shipley J., United kingdom Children's Cancer Study Group. United Kingdom Cancer Cytogenetics Group Cytogenetic abnormalities in 42 rhabdomyosarcoma: a United Kingdom Cancer Cytogenetics Group Study. Med Pediatr Oncol. 2001 Feb;36(2):259–267. doi: 10.1002/1096-911X(20010201)36:2<259::AID-MPO1063>3.0.CO;2-K. [DOI] [PubMed] [Google Scholar]
  12. Kelly K. M., Womer R. B., Sorensen P. H., Xiong Q. B., Barr F. G. Common and variant gene fusions predict distinct clinical phenotypes in rhabdomyosarcoma. J Clin Oncol. 1997 May;15(5):1831–1836. doi: 10.1200/JCO.1997.15.5.1831. [DOI] [PubMed] [Google Scholar]
  13. Li F. P., Fraumeni J. F., Jr, Mulvihill J. J., Blattner W. A., Dreyfus M. G., Tucker M. A., Miller R. W. A cancer family syndrome in twenty-four kindreds. Cancer Res. 1988 Sep 15;48(18):5358–5362. [PubMed] [Google Scholar]
  14. Li F. P., Fraumeni J. F., Jr Rhabdomyosarcoma in children: epidemiologic study and identification of a familial cancer syndrome. J Natl Cancer Inst. 1969 Dec;43(6):1365–1373. [PubMed] [Google Scholar]
  15. Marina N. Long-term survivors of childhood cancer. The medical consequences of cure. Pediatr Clin North Am. 1997 Aug;44(4):1021–1042. doi: 10.1016/s0031-3955(05)70543-5. [DOI] [PubMed] [Google Scholar]
  16. Martelli H., Oberlin O., Rey A., Godzinski J., Spicer R. D., Bouvet N., Haie-Meder C., Terrier-Lacombe M. J., Sanchez de Toledo J., Spooner D. Conservative treatment for girls with nonmetastatic rhabdomyosarcoma of the genital tract: A report from the Study Committee of the International Society of Pediatric Oncology. J Clin Oncol. 1999 Jul;17(7):2117–2122. doi: 10.1200/JCO.1999.17.7.2117. [DOI] [PubMed] [Google Scholar]
  17. Meister L. A., Meadows A. T. Late effects of childhood cancer therapy. Curr Probl Pediatr. 1993 Mar;23(3):102–131. doi: 10.1016/0045-9380(93)90019-9. [DOI] [PubMed] [Google Scholar]
  18. Oberlin O., Rey A., Anderson J., Carli M., Raney R. B., Treuner J., Stevens M. C., International Society of Paediatric Oncology Sarcoma Committee, Intergroup Rhabdomyosarcoma Study Group, Italian Cooperative Soft Tissue Sarcoma Group, German Collaborative Soft Tissue Sarcoma Group Treatment of orbital rhabdomyosarcoma: survival and late effects of treatment--results of an international workshop. J Clin Oncol. 2001 Jan 1;19(1):197–204. doi: 10.1200/JCO.2001.19.1.197. [DOI] [PubMed] [Google Scholar]
  19. Pappo A. S., Shapiro D. N., Crist W. M., Maurer H. M. Biology and therapy of pediatric rhabdomyosarcoma. J Clin Oncol. 1995 Aug;13(8):2123–2139. doi: 10.1200/JCO.1995.13.8.2123. [DOI] [PubMed] [Google Scholar]
  20. Raney Richard Beverly, Meza Jane, Anderson James R., Fryer Christopher J., Donaldson Sarah S., Breneman John C., Fitzgerald Thomas J., Gehan Edmund A., Michalski Jeff M., Ortega Jorge A. Treatment of children and adolescents with localized parameningeal sarcoma: experience of the Intergroup Rhabdomyosarcoma Study Group protocols IRS-II through -IV, 1978-1997. Med Pediatr Oncol. 2002 Jan;38(1):22–32. doi: 10.1002/mpo.1259. [DOI] [PubMed] [Google Scholar]
  21. Ruymann F. B., Maddux H. R., Ragab A., Soule E. H., Palmer N., Beltangady M., Gehan E. A., Newton W. A., Jr Congenital anomalies associated with rhabdomyosarcoma: an autopsy study of 115 cases. A report from the Intergroup Rhabdomyosarcoma Study Committee (representing the Children's Cancer Study Group, the Pediatric Oncology Group, the United Kingdom Children's Cancer Study Group, and the Pediatric Intergroup Statistical Center). Med Pediatr Oncol. 1988;16(1):33–39. doi: 10.1002/mpo.2950160109. [DOI] [PubMed] [Google Scholar]
  22. Sorensen Poul H. B., Lynch James C., Qualman Stephen J., Tirabosco Roberto, Lim Jerian F., Maurer Harold M., Bridge Julia A., Crist William M., Triche Timothy J., Barr Frederic G. PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the children's oncology group. J Clin Oncol. 2002 Jun 1;20(11):2672–2679. doi: 10.1200/JCO.2002.03.137. [DOI] [PubMed] [Google Scholar]
  23. Stiller C. A. Centralised treatment, entry to trials and survival. Br J Cancer. 1994 Aug;70(2):352–362. doi: 10.1038/bjc.1994.306. [DOI] [PMC free article] [PubMed] [Google Scholar]

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