Periosteal Ewing's sarcoma is a histologically typical Ewing's sarcoma arising in the periosteum with no involvement of the medullary canal or cancellous bone. We describe four cases in our experience and review the literature, recalling the usual computed tomography diagnostic criteria and the therapeutic consequences. Prognosis of periosteal Ewing's sarcoma is generally better than for ordinary Ewing's sarcoma. In order to avoid inoculating the canal, the operator must strictly avoid perforating the cortical during the biopsy. At surgical resection, cortical resection can be partial maintaining a continuous diaphysis; this should be examined as a possibility for young patients in order to avoid the problems encountered with massive reconstruction followed by chemotherapy.
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