Prognostic value of clinicopathologic characteristics including neuroectodermal differentiation in osseous Ewing's sarcoma family of tumors in children

Aims and background: The aim of the present study was to determine the rela tionship between clinico-pathologic parameters, including neuroectodermal d ifferentiation, and their imipact on survival in a series of pediatric pati ents with osseous tumors of the Ewing's sarcoma family admitted to the Pedi atric Department of the Istituto Nazionale Tumori of Milan, Methods: Seventy-three patients were enrolled. The variables analyzed were sex, age, site of primary tumor, serum lactate dehydrogenase (LDH) level at diagnosis, involvement of periosseous soft tissues by primary tumor, prese nce of metastatic disease, status of disease after the treatment plan, as w ell as the presence of mitoses, morphologic and immunocytochemical neural m arkers, and neuroendocrine markers in the primary tumor, Results: Neural an d neuroendocrine markers were not significantly associated with any of the other parameters, In the univariate analysis, significant risk factors rela ted to unfavorable outcome were elevated LDH, metastatic disease, lack of c omplete remission after treatment, presence of mitoses and of morphological neural markers: immunbcyto-chemical neural and neuroendocrine markers lack ed prognostic value, In the multivariate analysis, only LDH levels and the status of disease following the treatment were retained. Conclusions: LDH level at diagnosis might be a useful marker to identify di fferent risk levels; neuroectodermal differentiation might have no clear-cu t impact on the clinical management of osseous Swing's sarcoma family of tu mors.