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60 - Rhabdomyosarcoma

from Part VI - Oncology

Published online by Cambridge University Press:  08 January 2010

By
Dave R. Lal ,
Charles A. Sklar  and
Michael P. LaQuaglia
Edited by
Mark D. Stringer ,
Keith T. Oldham  and
Pierre D. E. Mouriquand
Dave R. Lal
Affiliation:
Department of Surgery and Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
Charles A. Sklar
Affiliation:
Department of Surgery and Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
Michael P. LaQuaglia
Affiliation:
Department of Surgery and Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
Mark D. Stringer
Affiliation:
University of Otago, New Zealand
Keith T. Oldham
Affiliation:
Children's Hospital of Wisconsin
Pierre D. E. Mouriquand
Affiliation:
Debrousse Hospital, Lyon
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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%.

Type
Chapter
Information
Pediatric Surgery and Urology
Long-Term Outcomes
 , pp. 782 - 798
Publisher: Cambridge University Press
Print publication year: 2006

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