Telangiectatic osteosarcoma: The St. Jude Children's Research Hospital's experience

BACKGROUND. Telangiectatic osteosarcoma (TOS) is a rare subtype of osteosarcoma (OS). The authors reviewed their experience with TOS to characterize its histologic, radiologic, and clinical features. METHODS. The authors reviewed records, pathology material, and imaging studies from all patients with TOS who were treated between 1978 and 2005 and compared their outcomes with the outcomes of patients with all other subtypes of high-grade osteosarcoma (OS). RESULTS. Among 323 patients with OS, 22 patients (6.8%) had TOS. Two additional patients who were treated in Chile on a recent OS trial were included. The median age at diagnosis of the 24 patients was 15.7 years. Four patients (17%) had metastatic disease, and 9 of 21 patients (43%) had pathologic fractures. Only 5 patients (who were treated after 1994) underwent limb-salvage surgery. Estimates of 5-year event-free survival (58.3% ± 11.9%) and overall survival (66.8% ± 11.6%) were similar to those for patients with other OS subtypes (P ≥ .85). The absence of local disease progression and chemotherapy with ≥3 agents that were active against OS were correlated with improved outcome (P < .005). The presence of a pathologic fracture was not associated with surgery type or patient outcome. CONCLUSIONS. TOS was associated with a high rate of pathologic fracture. With multimodality therapy, the outcome of patients with TOS was similar to that of patients with other high-grade OS subtypes. The absence of local disease progression and chemotherapy with ≥3 active agents were associated with a favorable outcome.