Key result
Intensive multiagent chemotherapy achieves 10-year disease-free survival in a pediatric metastatic alveolar soft-part sarcoma case.
Why the study?
Alveolar soft-part sarcoma is a rare malignant tumor for which adjuvant radiation and chemotherapy are generally considered ineffective.
Does intensive multiagent chemotherapy improve survival in a patient with Alveolar soft-part sarcoma and pulmonary metastases?
Case Report (n=1)
Does intensive multiagent chemotherapy improve survival in a patient with Alveolar soft-part sarcoma and pulmonary metastases?
Intensive multiagent chemotherapy may lead to prolonged disease-free survival in patients with metastatic alveolar soft-part sarcoma, challenging the notion that it is generally ineffective.
May support individualized chemotherapy trials in rare pediatric sarcomas; leaves open need for prospective validation before practice change.
Alveolar soft-part sarcoma (ASPS) is a very rare malignant tumor, usually of the extremities, and is seen most often in adolescents and young adults. Surgical excision of the primary and pulmonary metastases has resulted in prolonged survival in some patients, but adjuvant radiation and/or chemotherapy are generally thought to be ineffective. The authors describe a 13-year-old patient with ASPS of an extremity who presented with multiple bilateral pulmonary metastases at diagnosis. Following intensive multiagent chemotherapy, pulmonary metastases showed in vivo evidence of tumor death. The patient has remained disease-free for 10 years following treatment.
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Nickerson et al. (2004) conducted a case report in Alveolar soft-part sarcoma (ASPS) (n=1). Intensive multiagent chemotherapy was evaluated on Disease-free survival. Intensive multiagent chemotherapy resulted in in vivo evidence of tumor death and a 10-year disease-free survival in a 13-year-old patient with alveolar soft-part sarcoma and pulmonary metastases.
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