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Review
. 2022 May 31;14(4):35448.
doi: 10.52965/001c.35448. eCollection 2022.

Dedifferentiated Chondrosarcoma: A Case Series and Review of the Literature

Charles A Gusho  1 , Linus Lee  1 , Athan Zavras  1 , Zachary Seikel  1 , Ira Miller  2 , Matthew W Colman  1 , Steven Gitelis  1 , Alan T Blank  1
Affiliations
Review

Dedifferentiated Chondrosarcoma: A Case Series and Review of the Literature

Charles A Gusho et al. Orthop Rev (Pavia). .

Abstract

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.

Keywords: chondrosarcoma; dedifferentiated chondrosarcoma; outcomes; prognosis; survival.

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Conflict of interest statement

ATB: (BMJ Case Reports: Editorial or governing board; Clinical Orthopaedics and Related Research: Editorial or governing board; exparel/pacira: Stock or stock Options; Journal of Oncology Practice: Editorial or governing board; Journal of Surgical Oncology: ad hoc reviewer; Lancet - Oncology: Editorial or governing board; Musculoskeletal Tumor Society: Board or committee member; Onkos Surgical: Paid consultant; Pediatric Blood and Cancer: Editorial or governing board; Rare Tumors: Editorial or governing board; Rush Orthopedic Journal: Editorial or governing board; Swim Across America Cancer Research Grant: Research support); SG: (Onkos Surgical: Paid consultant; Stock or stock Options; USMI: Stock or Stock Options). MWC: (Alphatec Spine: IP royalties; Paid consultant; AO Spine North America: Board or committee member; Research support; Cervical Spine Research Society: Board or committee member; CSRS: Research support; DePuy, A Johnson & Johnson Company: Paid presenter or speaker; K2M: Paid presenter or speaker; Musculoskeletal Tumor Society: Board or committee member; North American Spine Society: Board or committee member; Orthofix, Inc.: Paid presenter or speaker; Spinal Elements: Paid consultant. All other authors have no pertinent financial disclosures or pertinent conflicts of interest.

Figures

Figure 1.
Figure 1.. Dedifferentiated chondrosarcoma of the distal femur on histology (A) demonstrating large, pleomorphic spindle cells adjacent to sheets of smaller cells with myxoid stroma similar to this patient’s original chondrosarcoma (hematoxylin-eosin, original magnification, x100). The gross resected specimen is also shown (B).
Figure 2.
Figure 2.. Preoperative imaging of dedifferentiated chondrosarcoma affecting the distal femur on (A) anteroposterior (AP) and (B) lateral X-ray views, and (C) axial T1-weighted magnetic resonance imaging. (D) Immediate postoperative AP radiograph of the distal femoral replacement reconstruction.
Figure 3.
Figure 3.. Overall survival (A) and recurrence-free survival (B) profiles of patients treated for dedifferentiated chondrosarcoma.
See this image and copyright information in PMC

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