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60 - Rhabdomyosarcoma
- from Part VI - Oncology
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- By Dave R. Lal, Department of Surgery and Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, NY, USA, Charles A. Sklar, Department of Surgery and Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, NY, USA, Michael P. LaQuaglia, Department of Surgery and Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
- Edited by Mark D. Stringer, Keith T. Oldham, Pierre D. E. Mouriquand
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- Book:
- Pediatric Surgery and Urology
- Published online:
- 08 January 2010
- Print publication:
- 09 November 2006, pp 782-798
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- Chapter
- Export citation
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Summary
Soft tissue sarcomas are the sixth most common malignancy of childhood, with rhabdomyosarcoma (RMS) by far the most frequent. In the United States, approximately 350 cases of RMS are diagnosed per year. Since 1975, the yearly incidence of RMS has remained stable at approximately 4 per 1 million children younger than 20 years of age.
Over the last 30 years, improved survival and decreased morbidity in treatment of RMS have been accomplished through collaborative clinical trials in both the United States and Europe. In the United States the Intergroup Rhabdomyosarcoma Study Group (IRSG) was established in 1972. Their mission has been to enroll all children diagnosed in North America into randomized prospective clinical trials. This has largely been accomplished with over 80% of North American children diagnosed with RMS enrolled in one of four completed IRSG studies. Since the inception of the IRSG, the overall survival for patients with RMS has improved from about 25% to over 70%. Much of this improvement has been the result of a multidisciplinary approach to rhabdomyosarcoma including surgeons, oncologists, and radiation oncologists. The IRSG (now called the Children's Oncology Group soft tissue sarcoma committee) continues to strive for improved survival with decreasing patient morbidity, and is currently accruing patients for its fifth trial (IRS-V).
Currently, orbital tumors have the best prognosis with a 5-year survival of approximately 95%.