Wide local excision is the primary therapy for NRSTSs. Every attempt is made to obtain negative tumor margins, which can be accomplished in 50%-80% of patients. If the initial surgery does not achieve pathologically negative margins, reexcision should be performed in order to obtain clear margins.
The mainstay of local control for sarcomas of the head and neck is aggressive surgical resection. These tumors may be difficult to remove with wide surgical margins. However, modern reconstruction techniques with vascularized flaps, free composite grafts, and rotation flaps assist in complete resection. Lesions in the extremities are usually completely resectable.
Dissection of the lymph nodes is not always warranted because of the infrequency of lymph node involvement in association with NRSTSs. Lymph node resection is warranted if the lymph nodes are enlarged on examination or scanning or if the tumor arises in an area near lymph nodes.
In children with metastatic disease involving isolated pulmonary metastasis, exploratory thoracotomy should be performed in an attempt to resect all gross disease.
Metastatic disease, disease of high metastatic potential, or large and unresectable primary tumors may require chemotherapy as part of the treatment plan. Chemotherapeutic agents that demonstrate the most activity against NRSTS include ifosfamide, cyclophosphamide, and doxorubicin. Other chemotherapeutic agents that have shown activity either alone or in combination are vincristine, etoposide, cisplatin, and dactinomycin. Ongoing clinical trials are under way to prospectively evaluate the exact role of chemotherapy in managing NRSTSs.
For more on the treatment of NRSTSs, read here.
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Cite this: Fast Five Quiz: Test Your Knowledge of Soft-Tissue Sarcomas - Medscape - Jan 03, 2018.