Abstract

Rhabdomyosarcoma is a malignant tumor that represents the most common form of pediatric soft tissue sarcoma. It forms part of the group of small round blue cell tumors of childhood, and has a constant annual incidence of 6 cases per 1,000,000 population per year. It presents in a bimodal distribution that correlates with the occurrence of the two major histological subtypes (embryonal and alveolar). RMS diagnosis has been recently enhanced by tumor biological markers and molecular differentiation diagnostic tools that have improved not only the updated classification based on risk stratification, but also the treatment approach based on clinical group.

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