Abstract
BACKGROUND: Clear cell chondrosarcoma (CCC), a rare subtype of chondrosarcoma, is generally a low-grade tumor that arises commonly in the epiphyses of the proximal femur or humerus. The spine is an uncommon site of CCC that most commonly involves the thoracic spine with the lumbar spine as the second most common predilection. METHODS: We report an extremely rare case of a 22-year-old female with a giant-sized CCC in the thoracolumbar region that severely compressed the spinal cord on that level. Tumor removal, spinal cord decompression, and stabilization were done, followed by high-dose radiotherapy. RESULTS: Histopathology demonstrates CCC with high cellularity, some hemorrhagic areas, and scanty mitotic figures. The patient had a significant back pain improvement. There were some recoveries of neurological function by the time of the last follow-up. CONCLUSIONS: This report highlights the unusual location of this rare tumor, the unusual size, and moreover, the age at presentation. Despite its low-grade behavior, CCC needs to be treated as a true malignancy with wide resection whenever feasible. Adjuvant radiotherapy should also be considered as part of the surgical treatment because of the risk of de-differentiation, recurrence, and malignancy, especially in unresectable cases or cases with incomplete resection.