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The results of adjuvant chemotherapy in the treatment of STSHN have, on the whole, been disappointing. Doxorubicin and ifosfamide, which are the

two most active agents, have reported response rates of only about 25 percent, and most of these responses are short-lived.67 A meta-analysis of 1,568 patients of extremity STS from 14 trials using doxorubicin-based adjuvant chemotherapy in localized resectable STS of adults reported a 6 percent absolute benefit in

Figure 17-9. A, A 34-year-old man presented with a tumor of the right upper neck that had increased in size progressively over the preceding months. Clinical and radiologic examination was consistent with fixation of the mass to the adjacent mastoid process and occipital bone. Biopsy of the mass had been reported to show a spindle-cell lesion. B, The patient underwent wide excision of the tumor with a posterolateral neck dissection.C, At operation, the tumor was grossly adherent to the adjacent occipital bone so that its outer table had to be resected to secure tumor-free deep margins of excision. Histopathologic examination of the specimen revealed low-grade myxoid fibrosar-coma. D, The surgical defect was reconstructed using a pedicled trapezius flap with an acceptable cosmetic result.

local control and only 4 percent in terms of overall survival from adjuvant chemotherapy.68 Chemotherapy may have a role in the management of distant metastases, but the relative inefficacy of currently used drugs is highlighted by the observation that as many as 30 to 40 percent of patients with extremity sarcomas who are controlled locally using multi-modality treatment will develop distant metastases despite preoperative chemotherapy.69

The T-12 chemotherapy protocol for pediatric extremity osteosarcoma (OS) at the MSKCC consists of preoperative high-dose methotrexate and adri-amycin to select patients who would benefit from postoperative adjuvant chemotherapy based on the histologic response to neoadjuvant treatment. The potential advantages of such therapy include reduction in tumor size and vascularity, increasing tumor-free surgical margins, elimination of micrometastases, and providing prognostic information. Unfortunately, OSs of the craniofacial skeleton in adults have not shown the same response and the use of chemotherapy in these tumors must be considered experimental.

Newer approaches such as high-dose chemotherapy with GM-CSF support, liposome-encapsulated anthracycline therapy and differentiation therapy for liposarcoma are being evaluated. There are about 32 ongoing clinical trials at this time on STS70 and although only a few of these involve head and neck sarcomas specifically, information from some of the trials may prove useful in the management of STSHN.

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