An analysis of 6 patients with periosteal osteosarcoma treated by the authors along with a review of 55 patients reported in the literature demonstrates that periosteal osteosarcoma is distinctly different from conventional osteosarcoma or periosteal chondrosarcoma. Periosteal osteosarcoma is a less aggressive tumor than conventional osteosarcoma. It is a relatively well‐differentiated chondroblastic osteosarcoma occurring on the surface of the long bones of the extremities. Three patients demonstrated frank medullary invasion of tumor, two grossly and one microsurgically. Patients treated with marginal resection had a 70% local recurrence rate. Patients receiving wide resection or primary amputation have survived longer with less recurrence of disease. Overall, 10 of 61 patients are dead with metastatic disease with a mean reported follow‐up of 6 years and 7 months. Adjunctive therapy has been of no demonstrable aid in terms of prolonging survival. Medullary extension of this tumor should not be used to exclude this diagnosis. The authors believe that the treatment of choice is wide resection without adjunctive chemotherapy.
The lesion we described is an osteoid producing mitotically active spindle cell soft tissue neoplasm, which has light and ultrastructural features of an osteosarcoma. By immunohistochemistry it marks with p30/32MIC2-directed antibodies but fails to react with antibodies associated with other soft tissue neoplasms. The precise histogenesis of the lesion is unknown, but failure to elicit a periosteal reaction makes the periosteum less likely to be the primary tissue of origin. The failure to demonstrate any radiologic or histologic evidence of involvement of the underlying bone leaves the soft tissue as the most reasonable site of origin, although the parosteal region cannot be completely excluded.
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