(1) Background: Extraskeletal osteosarcoma (ESOS) is a malignant tumor characterized by the production of bone or bone matrix by tumor cells without any continuity into the skeletal bones. The standard treatment for localized ESOS is wide resection; however, the effect of (neo)adjuvant chemotherapy remains unclear. To investigate the effect of (neo)adjuvant chemotherapy for localized ESOS, we conducted a systematic review of studies comparing the 5-year disease-free survival rate between patients who underwent surgery combined with (neo)adjuvant chemotherapy and those who underwent surgery alone. (2) Methods: Of the 210 studies identified by systematically searching the PubMed, Embase, and Cochrane Central Register of Controlled Trials databases, 12 were included in the final analysis. These 12 articles were not randomized controlled trials, but retrospective studies. In total, 761 patients with localized ESOS were included in this study. (3) Results: The 5-year disease-free survival rate was 47.9% (187 of 390 patients) in the surgery and (neo)adjuvant chemotherapy group and 40.4% (150 of 371 patients) in the surgery alone group. The overall pooled odds ratio was 1.23 (95% confidence interval, 0.69-2.19; p = 0.479) and the heterogeneity I2 was 37%. (4) Conclusions: The effect of adjuvant chemotherapy on localized ESOS seems to be limited. Therefore, routine use of adjuvant chemotherapy for localized ESOS should be avoided. However, further randomized controlled trials are required to confirm these results.
Keywords: chemotherapy; extraskeletal osteosarcoma; prognosis; surgery; survival.