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Patterns of treatment failure in infants with primitive neuroectodermal tumors who were treated on CCG-921: a phase
Theodore S Hong1, Minesh P Mehta, James M Boyett
1Department of Human Oncology, School of Medicine, University of Wisconsin, Madison, WI 53792, USA.
Purpose:
To analyze patterns of treatment failure in infants with primitive neuroectodermal tumors (PNETs) who were treated primarily with chemotherapy in a large multi-institutional study.
Materials And Methods:
Sixty-five prospectively staged patients with PNET confirmed by central pathology review, who were 18 months or younger were treated on Children's Cancer Group Study 921 (CCG-921) primarily with chemotherapy. Forty-six patients had posterior fossa (PF) primary tumors and 19 patients had supratentorial (ST) primaries. Patterns of sites of initial treatment failure were analyzed and compared to failure patterns of 180 older children who had PF-PNETs, and 44 older children with ST-PNETs who were treated on the same protocol.
Results:
The entire cohort of younger patients fared much worse than those older than 18 months. Cumulative 5-year relapse incidence (+/-SE) for younger patients with PF-PNETs was 64.5 +/- 8.9% for patients without metastases (M0) compared to 71.4 +/- 13.4% for patients with metastases (M+). The cumulative 5-year relapse incidences for younger patients with ST-PNETs were 64.3 +/- 13.7% for M0 patients compared to 100 +/- 33.3% for M+ patients. Relapses in these patients occurred within 2 years. The overall treatment failure rate was significantly higher for younger compared to older patients with PF-PNET and ST-PNET. There was no statistically significant difference in relapse patterns between patients with PF primary tumors and ST primaries when stratified by stage. There was no statistically significant difference in relapse patterns between M0 and M+ patients. All patients had a high risk of recurrence at primary tumor site. Younger patients who had PF primary tumors without metastasis at presentation were significantly more likely to relapse in PF than older patients. Younger patients were at significant risk of relapse in the spine, but none had it as the sole site of first relapse.
Conclusions:
Despite aggressive chemotherapy, younger children with PNETs have high rates of treatment failure and fare worse than high-risk, older patients with PF-PNETs, indicating the need to maximize local, regional, and systemic therapies.
Insights
Infants with primitive neuroectodermal tumors (PNETs) treated with chemotherapy show high relapse rates, faring worse than older children. This highlights the need for intensified local, regional, and systemic therapies for these young patients.
Area of Science:
- Pediatric Oncology
- Neuro-Oncology
- Cancer Treatment Efficacy
Background:
- Primitive neuroectodermal tumors (PNETs) are aggressive central nervous system malignancies.
- Treatment outcomes for infants with PNETs are often poorer compared to older children.
- Understanding treatment failure patterns is crucial for improving survival rates in pediatric PNET patients.
Purpose of the Study:
- To analyze treatment failure patterns in infants diagnosed with primitive neuroectodermal tumors (PNETs).
- To compare treatment failure patterns in young PNET patients (≤18 months) with older children treated on the same protocol.
- To identify specific sites and patterns of relapse in infants with PNETs.
Main Methods:
- A cohort of 65 infants (≤18 months) with PNETs treated with chemotherapy on Children's Cancer Group Study 921 was analyzed.
- Patients were stratified by primary tumor location: posterior fossa (PF) and supratentorial (ST).
- Failure patterns were compared to older pediatric patients with PF-PNETs and ST-PNETs.
Main Results:
- Younger patients (infants) exhibited significantly higher overall treatment failure rates compared to older children.
- Cumulative 5-year relapse incidence for infants with PF-PNETs was high, regardless of metastatic status (M0: 64.5%, M+: 71.4%).
- Infants with ST-PNETs also showed high relapse rates (M0: 64.3%, M+: 100%), with relapses occurring within 2 years. High recurrence risk at the primary tumor site was observed in all patients. Younger patients with PF primary tumors without metastasis were more likely to relapse in the PF. Younger patients had a significant risk of spinal relapse, though not as the sole site of first relapse.
Conclusions:
- Despite intensive chemotherapy, infants with PNETs demonstrate poor outcomes with high treatment failure rates.
- Younger PNET patients fare worse than high-risk, older pediatric patients, underscoring treatment challenges.
- There is a critical need to enhance local, regional, and systemic treatment strategies for infants with PNETs to improve survival.
