Rhabdomyosarcoma in children.

Rhabdomyosarcoma is a rare childhood cancer that affects only approximately 300 children per year in the United States. The purpose of this review is to provide the reader a greater understanding of the complex diagnosis, assessment and treatment of rhabdomyosarcoma in children. This review focuses on the new risk classification that is the foundation of all present rhabdomyosarcoma protocols developed by the Children's Oncology Group of the United States and Canada. The new risk classification of low, intermediate and high encompasses the staging and grouping categories that were previously utilized. This review also provides a complete list of diagnostic tests and imaging required to identify rhabdomyosarcoma in any body site. Rapid diagnosis and recognition of this rare disorder will facilitate long-term survival. Rhabdomyosarcoma today has an overall survival of 70%, depending on the site, and in orbital and other sites survival is as high as 90%. The treatment approaches that have led to this doubling in survival over the last 25 years are reviewed. For a practitioner, this review can be used as a reference when a child with a suspicious mass is encountered.