去分化软骨肉瘤：病例系列及文献综述
Dedifferentiated Chondrosarcoma: A Case Series and Review of the Literature.
作者信息
Gusho Charles A, Lee Linus, Zavras Athan, Seikel Zachary, Miller Ira, Colman Matthew W, Gitelis Steven, Blank Alan T
机构信息
Orthopedic Surgery, Rush University Medical Center.
Pathology, Rush University Medical Center.
出版信息
Orthop Rev (Pavia). 2022 May 31;14(4):35448. doi: 10.52965/001c.35448. eCollection 2022.
BACKGROUND
Dedifferentiated chondrosarcoma (DCS) is a rare and aggressive malignancy with a poor prognosis. The purpose of this investigation was to describe our treatment and outcomes of 16 DCS patients at our institution and provide a review of the current literature.
METHODS
This study was a retrospective review over a consecutive twenty-year period. Data including treatment details and outcomes were recorded.
RESULTS
A total of 16 cases from 2000 to 2018 were identified. The median age (IQR) was 62 years (52; 69) and the majority of DCS arose in the femur (50%, n=8) and pelvis (25%, n=4). Fourteen (88%) cases underwent limb salvage/wide margin resection (n=13) or intralesional surgery (n=1). For all DCS, the median survival (IQR) was 46 months (12; 140), with both a five and ten-year probability of 32.1% (95% CI, 7.3% to 57.0%). At study conclusion, 81.3% (n=13) were deceased and 18.7% (n=3) were alive.
CONCLUSIONS
Our findings confirm the poor prognosis of DCS patients, with a five-year estimate of 32%. Together with existing literature, our data might help enable future strategic recommendation of these patients.
背景
去分化软骨肉瘤（DCS）是一种罕见且侵袭性强的恶性肿瘤，预后较差。本研究的目的是描述我院16例DCS患者的治疗情况及结果，并对当前文献进行综述。
方法
本研究是一项为期20年的回顾性研究。记录了包括治疗细节和结果在内的数据。
结果
共确定了2000年至2018年期间的16例病例。中位年龄（四分位间距）为62岁（52；69），大多数DCS发生在股骨（50%，n = 8）和骨盆（25%，n = 4）。14例（88%）患者接受了保肢/广泛边缘切除（n = 13）或病灶内手术（n = 1）。所有DCS患者的中位生存期（四分位间距）为46个月（12；140），5年和10年生存率均为32.1%（95%可信区间，7.3%至57.0%）。研究结束时，81.3%（n = 13）患者死亡，18.7%（n = 3）患者存活。
结论
我们的研究结果证实了DCS患者预后较差，5年生存率估计为32%。结合现有文献，我们的数据可能有助于为这些患者制定未来的战略建议。
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