Malignant Cartilage-Forming Tumors

Abstract

Chondrosarcomas are heterogeneous matrix-producing cartilaginous neoplasms with variable clinical behavior. Subtypes include conventional (75%), dedifferentiated (10%), clear cell (2%), mesenchymal (2%), and periosteal chondrosarcoma (<1%). Tumor location and primary vs secondary also play a role. In conventional chondrosarcoma, histologic grading (I, II, and III) remains the gold standard for predicting recurrence and metastases. Due to the locally aggressive but overall nonmetastatic behavior, grade I chondrosarcomas (primary and secondary) of long and short tubular bones have been reclassified as atypical cartilaginous tumor. In this review, the pathologic features of malignant cartilage tumors are discussed with updates on recent genetic findings.

Keywords: Clear cell; Conventional; Dedifferentiated; Hey1-NCOA2 rearrangement; IDH1/2 mutations; Mesenchymal; Periosteal; Secondary.