Clinical Outcomes of Pediatric Soft-tissue Sarcoma in the Extremities

Soft-tissue sarcomas are rare malignancies in children and adolescents, representing only 7.4 % of cancer cases in children <20 years of age. They are categorized into rhabdomyosarcomas (RMS) and non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS). RMS of the extremities is associated with a poorer prognosis than RMS overall. Important negative prognostic factors are higher stage and clinical group, positive regional nodes, larger size, and greater tumor invasiveness. Overall survival for extremity RMS is around 70 % at five years, with significant variability based on clinical group. NRSTS is a heterogeneous group with frequent location in the extremities. Data are more limited in pediatric NRSTS, but general negative prognostic factors are extent of disease, tumor size, quality of resection, and grade. Survival ranges from 90 % for low-grade tumors to 15 % for patients presenting with metastatic disease. Further efforts to improve the outcome for extremity RMS and to research pediatric NRSTS in general are essential for future studies.