An institutional review of clear cell chondrosarcoma.

UNLABELLED: Clear cell chondrosarcoma is a rare bone neoplasm with a slow progressive clinical course and infrequent metastasis, but with a high local recurrence rate. We sought to ascertain the long-term outcome of patients with this neoplasm and to identify possible factors predicting survival. Sixteen patients with clear cell chondrosarcomas treated at one institution and who had long-term clinical followup were identified. All patients were treated by surgical resection of the tumor, which was classified as clear (> 2 mm) in 10 patients and marginal or intralesional in six patients. Three patients had local recurrence after a median of 1.7 years. Metastatic disease developed in four patients with a median time to diagnosis of 8.1 years. Ten-year overall survival of patients with clear cell chondrosarcomas was 89%, and disease-free survival was 68%. Patients with surgical resections comprising clear margins had longer disease-free survival compared with patients with marginal and intralesional tumors. Inadequate surgical resection of clear cell chondrosarcoma leads to risk of local recurrence and metastatic disease. The malignancy has a tendency to metastasize relatively late, therefore, followup of patients is necessary after the generally accepted 5-year period. LEVEL OF EVIDENCE: Prognostic study, Level III (case control study). See the Guidelines for Authors for a complete description of levels of evidence.