Rhabdomyosarcoma

Abstract

Rhabdomyosarcoma is a malignant tumor of mesenchymal origin. It is the most common pediatric soft tissue sarcoma, accounting for approximately 5 % of childhood cancers. It is the third most common extracranial solid tumor in the pediatric population with an estimated 350 new cases diagnosed each year in the United States. Rhabdomyosarcoma can develop in a variety of anatomic locations and there are two primary histologic types: embryonal rhabdomyosarcoma (ERMS) is more common in infancy and early childhood, while alveolar rhabdomyosarcoma (ARMS) generally presents in later childhood and adolescence and carries a worse prognosis than ERMS. Staging of RMS is based on initial physical examination and imaging. Clinical groups are determined after surgical resection and pathologic analysis. Low-, intermediate- or high-risk stratification is then based on staging and clinical grouping. Standard treatment is a multimodal approach that includes surgical resection, systemic chemotherapy, and radiotherapy. The mainstay of surgical treatment is complete resection with adequate margins and nodal sampling, often by sentinel lymph node mapping. Over the past several decades, cure rates have improved drastically from 25 % to more than 70 %; however, while low-risk patients have a good prognosis, metastatic disease is still associated with a very poor prognosis.