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Ewing tumors in infants
Henk van den Berg1, Uta Dirksen, Andreas Ranft
1Department of Pediatric Oncology, Emma Children Hospital, Academic Medical Center, University of Amsterdamm, The Netherlands. h.vandenberg@amc.uva.nl
Insights
Infants diagnosed with Ewing family tumors, often peripheral neuroectodermal tumors (PNETs), show similar outcomes to older patients. This suggests that typical poor prognostic factors do not impact infant outcomes for these rare childhood cancers.
Area of Science:
- Pediatric Oncology
- Rare Childhood Cancers
- Ewing Sarcoma Family of Tumors
Background:
- Malignancies in infancy are exceptionally rare, with Ewing tumors being particularly uncommon.
- Existing assumptions suggest a poor prognosis for infant malignancies.
Purpose of the Study:
- To investigate the outcomes of Ewing family tumors in infants.
- To determine if infants with these rare tumors face a poorer prognosis compared to older children.
Main Methods:
- A cohort of 14 infants (<12 months) with Ewing family tumors was identified from multiple international databases (CESS81, CESS86, EICESS92, EuroEwing99).
- Tumor characteristics, treatment modalities, and patient outcomes were analyzed.
Main Results:
- The majority of infant tumors were peripheral neuroectodermal tumors (PNETs), predominantly axial.
- Treatment protocols and dose reductions were comparable to older patients.
- Achieved 5-year event-free survival (EFS) of 65% and overall survival (OS) of 72%, similar to older age groups.
Conclusions:
- Infants with Ewing family tumors are typically diagnosed with PNETs, often presenting as axial tumors.
- The prognosis for infants with Ewing family tumors is comparable to that of older patients.
- Commonly accepted adverse prognostic factors did not significantly affect outcomes in this infant cohort.
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.
