[Rare forms of dedifferentiated chondrosarcomas: About 3 cases].

Abstract

Conventional chondrosarcoma is the most common primary malignant bone tumor in adults. Dedifferentiated chondrosarcomas represent 10 to 15% of conventional chondrosarcomas and constitute a high-grade subtype with a poor prognosis, with a 5-year survival rate of 24%. These tumors are characterized by a mutation in one of the isocitrate dehydrogenase (IDH) 1 or 2 genes in 70% of cases. Histological diagnosis requires a biphasic morphology combining a low-grade conventional chondrosarcoma component with a high-grade non-cartilaginous sarcomatoid component. In the literature, this dedifferentiated component most often corresponds to an osteosarcoma or an undifferentiated sarcomatous component. This article describes three cases of dedifferentiated chondrosarcomas: two with bone localization and one with laryngeal cartilaginous localization presenting a rare dedifferentiation component, either epithelial or rhabdomyosarcomatous, which may pose a diagnostic challenge, particularly in micro-biopsy samples. The mechanism of dedifferentiation is still poorly understood, and the origin of the dedifferentiated component remains controversial.