The impact of adjuvant therapy in extraskeletal osteosarcoma: A propensity score-matched analysis of 226 cases from the bone and Soft Tissue Tumor Registry in Japan.
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BACKGROUND: Extraskeletal osteosarcoma (ESOS) is a rare malignant soft tissue tumor for which optimal treatment strategies remain unclear. Although several retrospective studies have suggested the potential efficacy of adjuvant therapies, selection bias has limited the reliability of these findings. This study analyzes the largest ESOS cohort using the Japanese Bone and Soft Tissue Tumor (BSTT) Registry, employing propensity score matching to evaluate treatment outcomes while minimizing selection bias. METHODS: We retrospectively analyzed 226 ESOS cases from the BSTT Registry from 2006 to 2022. Patient characteristics, treatment modalities, and survival outcomes were evaluated using Kaplan-Meier analysis and Cox proportional hazards modeling. Propensity score matching was performed to assess the efficacy of adjuvant therapies while controlling for patient background factors. RESULTS: The 5-year overall survival rate was 53.6 %. In the unmatched cohort, tumor location (p = 0.012), tumor size (p = 0.014), and surgical treatment (p < 0.001) significantly influenced survival. After propensity score matching, adjuvant chemotherapy demonstrated a significant survival benefit (p = 0.044), while adjuvant radiotherapy showed no significant improvement (p = 0.951). No significant difference was observed between osteosarcoma-type and soft tissue sarcoma-type chemotherapy regimens (p = 0.213 after matching). CONCLUSIONS: This large cohort study, utilizing propensity score matching, provides evidence for the efficacy of adjuvant chemotherapy in ESOS treatment. While surgical resection remains the cornerstone of treatment, our findings support incorporating adjuvant chemotherapy into treatment strategies, regardless of regimen type. Prospective studies are needed to confirm these findings.