Establishment and characterization of NCC-dCS2-C1: a novel patient-derived cell line of dedifferentiated chondrosarcoma.

Dedifferentiated chondrosarcoma (dCS) is a rare, aggressive subtype of chondrosarcoma, characterized by an abrupt transition between a low-grade cartilaginous tumor and high-grade non-cartilaginous sarcoma. Treatment of dedifferentiated chondrosarcoma is limited by its high metastatic potential and poor response to chemotherapy and radiotherapy. Surgical resection remains the primary approach; however, recurrence and distant metastases substantially reduce survival rates. To provide a fundamental research resource, we aimed to establish a novel cell line of dCS. We successfully developed the NCC-dCS2-C1 cell line using surgically resected tumor tissue from a patient with dCS. This cell line harbors an IDH1 mutation (p.R132S) and exhibits complex copy number variants. NCC-dCS2-C1 cells exhibited constant proliferation in monolayer culture, spheroid formation in low-attachment plates, and migration. High-throughput screening of 221 anticancer drugs using NCC-dCS2-C1 identified three candidates, ixazomib, pazopanib, and ponatinib, that demonstrated low IC50 values, indicating their potential efficacy in treating dCS. We conclude that NCC-dCS2-C1 is a valuable tool for preclinical and basic research on dCS.