[Malignant peripheral neuroepithelial tumors in childhood]

The different therapy modalities and course of disease of 42 patients with a malignant peripheral neuroepithelial tumor are retrospectively analyzed. Therapy was completed in 31 children, 25 of whom had a primary localized tumor and 6 a disseminated neuroepithelioma. 17 of the children with a localized illness survive disease free in contrast to no survivor in the group with a disseminated tumor. The effective chemotherapy combining vincristine, adriamycin, ifosfamide and actinomycin D must be complemented by an efficacious local control because malignant peripheral neuroepithelioma tend to recur locally. The prospective analysis of newly diagnosed patients and a standardized therapy regimen will show if malignant peripheral neuroepithelioma represents a distinct tumor entity, different from a Ewings-Sarcoma.