BACKGROUND: The aim of this retrospective study is to analyze history and treatment outcomes of pathological fracture (PF) in dedifferentiated chondrosarcoma (DdChS) of the limbs..
METHODS: We retrospectively reviewed 175 adult patients with primary DdChS of the limbs.Disease-specific survival (OS) and local recurrence (LR) were analyzed.
RESULTS: Median age was 66 years (range, 29-91). Most DdChS (121, 69.1%) were localized in the femur. Forty-nine (28.0%) had metastasis at diagnosis; thirty-nine DdChS (22.3%) had a PF.OS rate was lower in patients with metastasis at diagnosis (8.6% Vs 41.0% at 10 years, p < 0.001). A similar OS was observed among patients with localized disease, whether with/without PF (p = 0.638), with/without chemotherapy (p = 0.543) and independently from the type of surgery (resection/amputation) (p = 0.877). Amputation reduces the risk of LR (80.0% vs 63.1% at 5 years, p = 0.039), particularly in the PF group..
CONCLUSION: Patients with metastases have a particularly poor prognosis in DdChS, but pathological fracture does not influence prognosis in terms of survival and local control. Initial curative resection is essential in order to reduce the chance of recurrences. Amputation might be an option in patients with localized disease and a PF to reduce the risk of LR..