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Updated: Sep 3, 2026

Modeling Osteosarcoma Using Li-Fraumeni Syndrome Patient-derived Induced Pluripotent Stem Cells
Published on: June 13, 2018
Li-Fraumeni syndrome presenting with temporomandibular chondroblastic osteosarcoma following recurrent glioma
Sujeeth Kumar Shetty1, Sonakshi Prakash1, Devika M Krishna2
1Oral and Maxillofacial Surgery, JSS Dental College and Hospital, Mysore, Karnataka, India.
Abstract:
A man in his 20s with recurrent glioma developed rapid-onset trismus and left temporomandibular swelling. Imaging revealed condylar destruction with a mineralised mass. Initial biopsy suggested chondroblastic osteosarcoma; germline testing confirmed Li-Fraumeni syndrome with a pathogenic TP53 variant. He underwent segmental resection with temporomandibular joint disarticulation, facial-nerve sacrifice and vascularised free fibular flap reconstruction. The case underscores the need for germline testing in young patients with multiple primary malignancies and adherence to oncological principles despite challenging anatomy.
