Background: High-grade surface osteosarcoma is a rare variant of surface osteosarcoma and is associated with a poor prognosis.
Methods: This retrospective study includes all cases of high-grade surface osteosarcoma filed at the Rizzoli Institute. A complete clinical, histologic, and radiologic review was performed of 25 cases.
Results: There were 19 males and 6 females with an average age of 21 years. All tumors were localized at the lower limb and the most frequently involved segments were the femoral and the tibial diaphysis. All lesions demonstrated the histologic aspect of a high-grade osteosarcoma, 20 were osteoblastic, and 5 were chondroblastic. The circumferential involvement was on average 53% and in approximately half of the cases medullary canal involvement was detected. Nineteen patients underwent a combination of surgery and chemotherapy for treatment of their initial lesion, whereas 5 patients were managed with surgery alone. The average follow-up was 9 years and the overall 5-year survival rate was 82%.
Conclusions: High-grade surface osteosarcoma is an extremely rare subtype of osteosarcoma. The current series demonstrates good overall survival for patients with this tumor. Metastatic disease and limb-sacrificing surgery were associated with a worse prognosis. Wide surgical excision and chemotherapy might improve the outcome.