University of Bologna, Italy; AOU Sant'Orsola Malpighi, Bologna, Italy.
IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy.
J Orthop Sci. 2021 May;26(3):473-477. doi: 10.1016/j.jos.2020.04.013. Epub 2020 Jun 18.
The aim of this retrospective study is to analyze history and treatment outcomes of pathological fracture (PF) in dedifferentiated chondrosarcoma (DdChS) of the limbs..
We retrospectively reviewed 175 adult patients with primary DdChS of the limbs.Disease-specific survival (OS) and local recurrence (LR) were analyzed.
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..
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..
本回顾性研究旨在分析四肢去分化软骨肉瘤（DdChS）病理性骨折（PF）的病史和治疗结果。
我们回顾性分析了 175 例成人肢体原发性 DdChS 患者。分析了疾病特异性生存（OS）和局部复发（LR）。
中位年龄为 66 岁（范围 29-91 岁）。大多数 DdChS（121 例，69.1%）位于股骨。49 例（28.0%）诊断时已有转移；39 例 DdChS 发生 PF。诊断时转移的患者 OS 率较低（10 年时为 8.6%比 41.0%，p<0.001）。局部疾病患者无论是否发生 PF（p=0.638）、是否接受化疗（p=0.543）以及手术类型（切除/截肢）是否不同（p=0.877），OS 相似。截肢可降低 LR 风险（5 年时为 80.0%比 63.1%，p=0.039），特别是在 PF 组中。
转移患者的 DdChS 预后特别差，但 PF 对生存和局部控制的预后无影响。初始根治性切除对于降低复发的机会至关重要。对于局部疾病和 PF 的患者，截肢可能是降低 LR 风险的一种选择。
