• PURPOSE
    • Periosteal chondrosarcoma (PCS) is the rarest subtype of chondrosarcoma and is recognized as a low-grade malignant tumour, reported to have an 88% ten year overall survival rate. The relationship between surgical margins and clinical outcome is inconsistent; some authors claim that PCS can be successfully treated with marginal resection and others report more local recurrence and distant metastasis with marginal compared to wide resection. This study was intended to report the treatment and prognosis of localized PCS patients from the Japanese National Bone and Soft Tissue Tumor Registry database and to perform a systematic review of the literature to determine the relationship between surgical margins and rates of local recurrence, distant metastasis, and mortality.
  • METHODS
    • Twenty-three patients with pathologically-diagnosed PCS between 2006 and 2022 from the Japanese National Bone and Soft Tissue Tumor Registry database were retrospectively analyzed. Of the 105 studies found through a systematic search using Pubmed, Embase, and Cochrane Central Register of Controlled Trials databases, nine studies were ultimately included.
  • RESULTS
    • The local recurrence rate after R0 resection was 2.5% (3/118 patients) and after R1 resection was 33.3% (11/33 patients) (p < 0.001). The distant metastasis rate in the R0 resection group was 5.9% (7/118 patients) and in the R1 resection group was 27.3% (9/33 patients) (p = 0.010). Mortality following R0 resection was 4.2% (5/118 patients) but after R1 resection was 18.2% (6/33 patients) (p = 0.040).
  • CONCLUSION
    • Wide resection may be necessary for localized PCS to prevent local recurrence and distant metastasis and improve survival.