Background: Malignancies in infancy are extremely rare. Ewing tumors are hardly ever noted in these children. Since it is generally assumed that malignancies in infancy have an extremely poor outcome, we wanted to investigate whether this was also the case in Ewing tumors.
Procedure: We identified in the Munster data bases of CESS81, CESS86, EICESS92 and EuroEwing99 14 children <12 months of age with a tumor of the Ewing family. Numbers of girls and boys were equal.
Results: All infants had axial tumors, including pelvic primaries; 80% of the tumors were <200 ml. An uncommon pathology distribution was noted; the majority were peripheral neuroectodermal tumors, only two atypical Ewing, one classical Ewing, and one unspecified PAS positive small round blue cell soft tissue sarcoma was found. Three patients had metastatic disease at initial diagnosis. Treatment modalities were comparable with patients of older age. The number of cytostatic courses ranged from 6 to 15. Dose reductions were limited, ranged from 73% to 90%. Outcome results were similar to those in patients of older ages (5-year EFS 65%, OS 72%).
Conclusions: Infants with Ewing family tumors are in the majority of cases PNETs and are predominantly axial tumors. Outcome is similar to patients with Ewing tumors at older ages. Generally accepted adverse prognostic factors did not influence outcome.
(c) 2008 Wiley-Liss, Inc.