Extraskeletal Osteosarcoma: Analysis of Outcome of a Rare Neoplasm

[摘要] Purpose.Extraskeletal osteosarcoma represents an unusual soft-tissuesarcoma that historically is reported to carry an exceptionally poor prognosis.The objectivesof this study were to use a prospectively gathered sarcoma database to test the prevailingclinical bias and more accurately describe the natural history, characterize the prognosticfeatures, estimate survival and evaluate treatment strategies for this unusual sarcoma.Patients and methods.From a large database of nearly 4000 sarcomasat a single institution, 15 patients with pathologically confirmed extraskeletal osteosarcomawere analysed.Results and discussion.Extraskeletal osteosarcoma usually occurs as alarge, deep, high-grade lesion in the lower extremity of older patients. Overall anddisease-specific survival at 5 years was 50%, with a median follow-up of 35 months (range3– 200 months). Use of adjuvant chemotherapy or radiation therapy did not appear to influence survival, but an effect may have been missed by the relatively low numbers in eachgroup.When matched to a comparable group of patients with stage III extremity sarcomas,there was no significant difference in overall or disease-specific survival between groups.Treatment for extraskeletal osteosarcoma should follow established guidelines for treatmentof soft-tissue sarcomas, with the decision regarding adjuvant therapy to be based onindividual risk factors.

[学科分类] 肿瘤学