Abstract
Chondrosarcomas are the most common malignant tumors of the cartilage, are seen predominantly in adults, and have varied clinical behavior. The majority of them affect the medullary canal of long bones and pelvic bones. The prognosis of chondrosarcoma is closely related to histological grading; however, the grading is subject to interobserver variability. Conventional chondrosarcomas are overall considered to be chemotherapy- and radiation-resistant, resulting in limited treatment options. The majority of advanced conventional chondrosarcomas are treated with chemotherapy without any survival benefit. Recent studies have evaluated molecular genetic findings which have improved the understanding of chondrosarcoma biology. Newer therapeutic targets are desperately needed. In this review article, we explore ongoing clinical trials evaluating novel ways of treating advanced conventional chondrosarcoma.
Highlights
Chondrosarcoma is a rare malignant tumor which forms cartilaginous matrix
Chondrosarcomas arising de novo are termed primary chondrosarcomas, while those developing in pre-existing benign cartilaginous tumors such as osteochondroma or enchondroma are referred to as secondary chondrosarcomas
Treatment with palbociclib led to attenuation of Cyclin-dependent Kinase (CDK) 4, thereby inhibiting chondrosarcoma cell viability via regulation of the CDK 4/RB signaling pathway
Summary
Chondrosarcoma is a rare malignant tumor which forms cartilaginous matrix. The estimated overall incidence is 1 per 200,000/year and accounts for 20–30% of primary malignant bone tumors [1]. The five-year survival rate for patients with grade 1 chondrosarcoma is approximately 83% and the major cause of death in these patients is from local recurrence typically involving the pelvis and shoulder girdle, which is difficult to manage surgically [10,11]. Current chemotherapy recommendations include cisplatin and doxorubicin, extrapolated from recommended osteosarcoma treatment regimens Their use is based on retrospective studies [15,16] and one prospective study [17] consisting of a small number of patients. Five-year overall survival for stage IV patients was 28.2% with chemotherapy and 31.2% without chemotherapy (p = 0.366) This outlines an urgent need for more effective and novel treatment strategies for the management of this cancer.
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