Abstract

Chondrosarcoma is the most common bone sarcoma in adults. Conventional chondrosarcoma, the commonest histological subtype, is largely resistant to anthracycline-based chemotherapy. There have been anecdotal reports of durable clinical benefit with antiangiogenic agents in this disease. A retrospective search of patients treated at three sarcoma referral centers was performed to identify patients with advanced chondrosarcoma treated with antiangiogenic agents. The aim of this study was to evaluate the efficacy and safety of antiangiogenic agents in advanced chondrosarcoma. Ten patients were identified; seven with conventional, one each with clear cell, extraskeletal mesenchymal chondrosarcoma and extraskeletal myxoid chondrosarcoma. The median progression-free survival for patients with conventional and clear cell sarcoma was 22.6 months. Median overall survival has not been met. Antiangiogenic therapy was well tolerated in this series of patients. Our retrospective data suggest that antiangiogenic therapy can provide prolonged clinical benefit in advanced chondrosarcoma patients. Further prospective trials are required to precisely define the role of this class of agent in advanced chondrosarcoma.

Highlights

  • Chondrosarcoma is a malignant tumor characterized by the production of cartilage matrix and displaying varied histopathology and clinical behavior [1]

  • In order to better understand the clinical course of patients with advanced chondrosarcoma treated, we provide case descriptions below of the two patients with the most durable responses to antiangiogenic therapy

  • Our retrospective study shows that patients with advanced/ metastatic chondrosarcoma can obtain prolonged clinical benefit with antiangiogenic therapy which was well tolerated

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Summary

Introduction

Chondrosarcoma is a malignant tumor characterized by the production of cartilage matrix and displaying varied histopathology and clinical behavior [1]. Chondrosarcomas are the most common bone sarcomas in adults and following myeloma and osteosarcoma, the third most frequent primary malignant tumor of bone. There are several histological subtypes, including conventional, mesenchymal, dedifferentiated and clear cell. The conventional subtype accounts for approximately 85% of all chondrosarcomas. Extraskeletal mesenchymal and extraskeletal myxoid chondrosarcoma, do not carry typical features of chondrosarcoma. Extraskeletal mesenchymal chondrosarcoma carry mixed features of Ewing sarcoma and chondrosarcoma, while extraskeletal myxoid chondrosarcoma is a soft tissue sarcoma

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