Extra-axial chordoma of the distal tibia incidentally discovered during fixation of a presumed pathologic fracture

Chordomas are rare malignant tumors of notochordal origin that typically arise along the axial skeleton. Extra-axial chordomas involving the appendicular skeleton are exceedingly uncommon and may present diagnostic challenges due to nonspecific clinical and radiographic features. We report a case of a 59-year-old woman who sustained a low-energy distal tibial fracture and proximal fibula fracture and was found intraoperatively to have an extra-axial chordoma of the distal tibia initially presumed to be a benign cystic lesion. The tumor was excised at the time of fracture fixation, and pathologic analysis confirmed the diagnosis of extra-axial chordoma. This case highlights a highly unusual traumatic presentation of a distal appendicular chordoma and emphasizes the importance of maintaining suspicion for occult pathology in atypical cortical lesions encountered during orthopedic trauma surgery.