Abstract
Rhabdomyosarcoma (RMS) is a malignant tumor that represents the most common form of pediatric soft tissue sarcoma. It arises from mesenchymal origin and forms part of the group of small round blue cell tumors of childhood. It has a constant annual incidence of 4.5 cases per 1,000,000 children. The known histological diagnosis of the two major subtypes (embryonal and alveolar) 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 the clinical group. Ewing sarcoma (ES) is a round cell tumor, highly malignant and poorly differentiated that is currently the second most common malignant bone tumor in children. In rare instances, it develops from an extraskeletal origin, classified as extraosseous Ewing sarcoma (EES). We provide an updated, evidence-based and comprehensive review of the molecular diagnosis, clinical and diagnostic approach and a multidisciplinary medical and surgical management according to the latest standard of care for the treatment of pediatric RMS and EES.
Highlights
Rhabdomyosarcoma (RMS) is a malignant tumor that represents the most common form of pediatric soft tissue sarcoma
This bimodal distribution correlates with the occurrence of the two primary histologic subtypes of RMS: Embryonal rhabdomyosarcoma (ERMS) in early childhood, which typically presents in the head, neck and genitourinary (GU) regions; and alveolar (ARMS) for the later childhood and adolescent years which is commonly located in the trunk and extremities
Pre-treatment re-excision (PRE) of RMS should be considered in cases where the surgical margins are positive, a non-oncologic excision was performed, or when only a biopsy was taken, if the surgeon feels that a complete resection with negative margins is feasible prior to starting chemotherapy
Summary
Rhabdomyosarcoma (RMS) is the most common form of pediatric soft tissue sarcoma accounting for 5% of all childhood cancers [1]. More than 80% of cases are diagnosed before age 14 [4] This bimodal distribution correlates with the occurrence of the two primary histologic subtypes of RMS: Embryonal rhabdomyosarcoma (ERMS) in early childhood, which typically presents in the head, neck and genitourinary (GU) regions; and alveolar (ARMS) for the later childhood and adolescent years which is commonly located in the trunk and extremities. ERMS is (IHC) stains used to identify RMS include MyoD1, desmin, myogenin, and muscle specific actin. ERMS includes: Botryoid, Historically, RMSis is further subdivided according to histologic spindle cell, and dense patterns. Spindle cell histology is commonly found in paratesticular lesions while botryoid tumors better prognosis. (c) Immunohistochemical for myogenin highlights of the overwhelming of infiltrating tumor cells, densely contrast, nuclei ofnuclei the collagenous connective tissue in majority of infiltrating tumor cells, packed. Of the collagenous connective the background remain appropriately negative.negative
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