Chondrosarcoma organoids reveal SHH pathway activation driven by PTCH1 and BCOR alterations.

ABSTRACT: Chondrosarcoma is the second most common malignant bone tumor, characterized by the production of cartilaginous matrix and a high degree of resistance to conventional therapies such as chemotherapy and radiotherapy. Effective treatment options remain limited, highlighting the urgent need for preclinical models to explore novel therapeutic approaches. This study aimed to establish patient-derived organoid (PDO) models of chondrosarcoma and to investigate their utility in elucidating molecular mechanisms and drug responses. Chondrosarcoma specimens were collected from patients and cultured using a modified air-liquid interface (ALI) organoid method. The resulting PDO were serially expanded in vitro and transplanted into NOD-SCID IL2Rgnull mice for in vivo validation. Histological and genetic

SUBMITTER: Takami H 

PROVIDER: S-EPMC12830980 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature