Small round blue cell tumors in bone: prognostic factors correlated to Ewing's sarcoma and neuroectodermal tumors

Ewing's sarcoma and peripheral neuroectodermal tumors are the most common small round blue cell tumors of bone. Accurate prognostic factors are required to define guidelines to standardize the treatment modalities and to adapt these modalities to the potential evolution of the disease. The various factors that have emerged in the literature as influences on the outcome of patients with Ewing's sarcoma or peripheral neuroectodermal tumors of bone are considered in this review. The presence of metastases at the clinical onset of the disease represents the most adverse prognostic factor. For nonmetastatic patients, axial location appears to be the most unfavorable factor, despite initial tumor volume, by showing that the tumor burden could be a more appropriate indicator of patient outcome. The importance of a local control of the disease by surgery has been emphasized, and the value of the histopathologic evaluation of the response to chemotherapy has been stressed.