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BACKGROUND: Periosteal osteosarcoma is a rare variant of osteosarcoma. Wide surgical removal is the mainstay of treatment, but controversy remains about the role of chemotherapy. The objective of this study was to review and analyze the clinical and treatment-related factors that influence the survival of patients with periosteal osteosarcoma who received treatment in a single institution. METHODS: Thirty-three patients with periosteal osteosarcoma (19 males and 14 females) with a median age of 16 years (range, ages 6-32 years) underwent surgery (32 patients) and received radiotherapy (1 patient). Chemotherapy was received according to different regimens for high-grade osteosarcoma by 14 patients who had grade 3 tumors. RESULTS: The 10-year overall survival rate was 84%. The only patient who did not undergo surgery died of disease after 9 months; for the remaining 32 patients the 10-year disease-free survival rate was 65%. Survival was not influenced by the receipt of chemotherapy. The patients who received chemotherapy had a 10-year overall survival rate of 86%, and those who received only local treatment had an overall survival rate of 83% (P ¼ .73). CONCLUSIONS: The authors’ experience indicated that the treatment of periosteal osteosarcoma requires only wide surgical removal of the tumor and that adjuvant chemotherapy does not improve survival. Cancer 2010;000:000–000. V C 2010 American Cancer Society.

BACKGROUND Risk factors for local recurrence (LR) after osteosarcoma, such as surgical margins and histologic response to preoperative treatment, have been well documented, whereas the outcome for patients who locally recur has not been well established yet. METHODS Retrospective analysis of the management and outcome of 44 patients who developed LR after treatment of osteosarcoma of the extremities with neoadjuvant chemotherapy was performed in a single institution between 1983 and 1999. RESULTS In 24 patients (54.5%), LR was the first sign of recurrence; in 8 patients (18.2%) LR followed systemic recurrence and in 12 patients (27.3%), the 2 events, local and systemic recurrence, were concurrent. Of the 44 patients, 26 with local recurrences were free of disease, but only 5 were long-term event-free survivors, and 39 patients developed further recurrences: 37 died of the tumor and 2 were alive with uncontrolled disease at the time of last follow-up. The 5-year disease-free survival rate after the last recurrence was 15.9%; it was 25.9% for patients who achieved remission and 0% for the others. The only prognostic factor identified for post-LR disease-free survival was the presence of systemic recurrence at the time of diagnosis of LR or before (5-year postrecurrence event-free survival rate of 29.1% for patients without metastases at the time of local recurrence vs. 0% for those with metastases; P = .02). CONCLUSIONS These results confirm that patients with osteosarcoma of the extremities who develop LR are at a very significantly high risk of developing metastatic disease and dying of the tumor. Cancer 2006. © 2006 American Cancer Society.