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. 2019 Dec 13:2019:9069272.
doi: 10.1155/2019/9069272. eCollection 2019.

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

Ruoyu Miao  1 , Edwin Choy  2   3 , Kevin A Raskin  3   4 , Joseph H Schwab  3   4 , Gunnlaugur Petur Nielsen  3   5 , Vikram Deshpande  3   5 , Ivan Chebib  3   5 , Thomas F DeLaney  1   3 , Francis J Hornicek  3   4   6 , Gregory M Cote  2   3 , Yen-Lin E Chen  1   3
Affiliations

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

Ruoyu Miao et al. Sarcoma. .

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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Conflict of interest statement

The authors report no conflicts of interest regarding the publication of this article.

Figures

Figure 1
Figure 1
Anatomic sites of dedifferentiated chondrosarcoma.
Figure 2
Figure 2
Kaplan–Meier curves. (a) Overall survival and (b) progression-free survival in patients with dedifferentiated chondrosarcoma with and without metastasis at diagnosis. (c) Local relapse-free survival in patients with nonmetastatic dedifferentiated chondrosarcoma according to surgical margin. (d) Metastasis-free survival in patients with nonmetastatic dedifferentiated chondrosarcoma according to the size of dedifferentiation.
See this image and copyright information in PMC

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