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.
Highlights
Dedifferentiated chondrosarcoma (DDCS) is a type of cartilaginous tumor that is comprised of two distinct components: (1) low-grade chondrogenic components and (2) high-grade noncartilaginous sarcoma
undifferentiated pleomorphic sarcoma (UPS) dedifferentiation was reported to be related with poorer outcomes [3]; other studies did not reveal any difference in the clinical outcome with different types of the dedifferentiated component [6, 16]
We queried the database for patients who were diagnosed with DDCS that was confirmed by pathology review at our institution by expert sarcoma pathologists and were treated in our institution between 1993 and 2017
Summary
Dedifferentiated chondrosarcoma (DDCS) is a type of cartilaginous tumor that is comprised of two distinct components: (1) low-grade chondrogenic components and (2) high-grade noncartilaginous sarcoma. Twenty-three patients (31.9%) presented with distant metastasis, and 3 (4.2%) of them had regional lymph node involvement. 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 without metastasis at diagnosis, 17 (34.7%) developed local recurrence
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