Treatment of Nonmetastatic Childhood Soft Tissue Sarcoma
Some of these tumors have been treated with other therapies and these selected tumors and treatments are discussed below.
Alveolar soft part sarcoma
The standard approach is complete resection of the primary lesion. If complete excision is not feasible, radiation therapy should be administered. The value of adjuvant chemotherapy in completely resected ASPS remains unproven, particularly because patients with unresected or metastatic tumors failed to respond to chemotherapeutic agents frequently used to treat soft tissue sarcomas. Patients with ASPS may relapse several years after a prolonged period of apparent remission. The role of adjuvant chemotherapy in children with this malignancy has not been tested. Because these tumors are rare, all children with ASPS should be enrolled in prospective clinical trials.
Treatment options under clinical evaluation for alveolar soft part sarcoma
The following are examples of national and/or institutional clinical trials that are currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.
Clear cell sarcoma
Treatment for clear cell sarcoma is primarily surgical with radiation therapy for uncertain or involved margins. Antisarcoma chemotherapy is rarely effective. In one series, clear cell sarcoma demonstrated a propensity to metastasize to regional lymph nodes (12%-43%).
Desmoplastic small round cell tumor
Complete resection of this tumor is rarely possible at diagnosis, but greater than 90% tumor resection after neoadjuvant chemotherapy has been shown to be a prognostic factor for overall survival. Therefore, treatment may include chemotherapy, surgery, and radiation therapy. Multiagent chemotherapy analogous to that used for sarcomas has been used, as well as total abdominal radiotherapy.[39,40,41,42,43]