Prognostic Factors in Dedifferentiated Chondrosarcoma: A Retrospective Analysis of a Large Series Treated at a Single Institution.

Q2 Medicine Sarcoma Pub Date : 2019-12-13 eCollection Date: 2019-01-01 DOI:10.1155/2019/9069272

Ruoyu Miao, Edwin Choy, Kevin A Raskin, Joseph H Schwab, Gunnlaugur Petur Nielsen, Vikram Deshpande, Ivan Chebib, Thomas F DeLaney, Francis J Hornicek, Gregory M Cote, Yen-Lin E Chen

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引用次数: 25

Abstract

Background: Dedifferentiated chondrosarcomas (DDCSs) are highly malignant tumors with a dismal prognosis and present a significant challenge in clinical management.

Methods: In an IRB approved retrospective protocol, we identified 72 patients with DDCS treated at our institution between 1993 and 2017 and reviewed clinicopathological characteristics, treatment modalities, and outcomes to analyze prognostic factors.

Results: Femur (44.4%), pelvis (22.2%), and humerus (12.5%) were most commonly involved sites. Twenty-three patients (31.9%) presented with distant metastasis, and 3 (4.2%) of them also had regional lymph node involvement. The median overall survival (OS) was 13.9 months. On multivariate analysis, pathological fracture, larger tumor size, lymph node involvement, metastasis at diagnosis, extraosseous extension, and undifferentiated pleomorphic sarcoma component correlated with worse OS, whereas surgical resection and chemotherapy were associated with improved OS. For progression-free survival (PFS), pathological fracture and metastasis at diagnosis showed increased risk, while chemotherapy was associated with decreased risk. Among patients who received chemotherapy, doxorubicin and cisplatin were significantly associated with improved PFS but not OS. Among patients without metastasis at diagnosis, 17 (34.7%) developed local recurrence. Thirty-one (63.3%) developed distant metastases at a median interval of 18.1 months. On multivariate analysis, R1/R2 resection was related with local recurrence, while macroscopic dedifferentiated component was associated with distant metastasis.

Conclusions: The prognosis of DDCS is poor. Complete resection remains a significant prognostic factor for local control. Chemotherapy with doxorubicin and cisplatin seems to have better PFS. More prognostic, multicenter trials are warranted to further explore the effectiveness of chemotherapy in selected DDCS patients.

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去分化软骨肉瘤的预后因素:在单一机构治疗的大系列回顾性分析。

背景:去分化软骨肉瘤(Dedifferentiated chondrosarcoma, DDCSs)是一种预后不佳的高度恶性肿瘤，在临床治疗中面临着重大挑战。方法:在IRB批准的回顾性方案中，我们确定了1993年至2017年在我们机构治疗的72例DDCS患者，并回顾了临床病理特征、治疗方式和结果，以分析预后因素。结果:股骨(44.4%)、骨盆(22.2%)和肱骨(12.5%)是最常见的受累部位。23例(31.9%)出现远处转移，3例(4.2%)伴有局部淋巴结受累。中位总生存期(OS)为13.9个月。在多因素分析中，病理性骨折、较大肿瘤大小、淋巴结累及、诊断时转移、骨外延伸和未分化多形性肉瘤成分与较差的OS相关，而手术切除和化疗与改善OS相关。对于无进展生存期(PFS)，诊断时病理性骨折和转移的风险增加，而化疗的风险降低。在接受化疗的患者中，阿霉素和顺铂与PFS的改善显著相关，但与OS无关。在诊断时无转移的患者中，17例(34.7%)发生局部复发。31例(63.3%)发生远处转移，中位时间间隔为18.1个月。在多因素分析中，R1/R2切除与局部复发有关，而宏观去分化成分与远处转移有关。结论:DDCS预后较差。完全切除仍然是局部控制的重要预后因素。用阿霉素和顺铂化疗似乎有更好的PFS。需要更多的预后、多中心试验来进一步探讨化疗在选定的DDCS患者中的有效性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Sarcoma Medicine-Radiology, Nuclear Medicine and Imaging

CiteScore

5.00

自引率

0.00%

发文量

审稿时长

14 weeks

期刊介绍： Sarcoma is dedicated to publishing papers covering all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology and the clinical sciences of epidemiology, surgery, radiotherapy and chemotherapy. High-quality papers concerning the entire range of bone and soft tissue sarcomas in both adults and children, including Kaposi"s sarcoma, are published as well as preclinical and animal studies. This journal provides a central forum for the description of advances in diagnosis, assessment and treatment of this rarely seen, but often mismanaged, group of patients.