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Intracranial germ cell tumors: a retrospective study of 44 children
Tang-Her Jaing1, Huei-Shyong Wang, Iou-Jih Hung
1Division of Hematology, Chang Gung Children's Hospital, Taoyuan, Taiwan.
Insights
Histologic tumor type significantly impacts outcomes for pediatric intracranial germ cell tumors. Germinomas show superior survival rates compared to nongerminomatous types, suggesting tailored treatment approaches are crucial.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Tumor Biology
Background:
- Intracranial germ cell tumors (GCTs) are rare in pediatric patients.
- Understanding prognostic factors is vital for optimizing treatment strategies.
Purpose of the Study:
- To identify factors influencing prognosis and treatment outcomes in patients under 17 with primary intracranial GCTs.
- To evaluate the impact of tumor histology on survival and event-free rates.
Main Methods:
- Retrospective analysis of 44 pediatric patients diagnosed with intracranial GCTs over 16 years.
- Kaplan-Meier survival estimates were used for univariate analysis of prognostic factors.
- Comparison of survival rates between germinomas and nongerminomatous GCTs.
Main Results:
- Histologic tumor type was the only significant prognostic factor (P < 0.005).
- Five-year overall and event-free survival rates were 92.6% for germinomas versus 47.3% and 42.1% for nongerminomatous GCTs, respectively.
- Radiation involving the spinal axis showed limited benefit for intracranial GCTs, though radiotherapy improved outcomes for germinomas.
Conclusions:
- Histology is a critical determinant of outcome in pediatric intracranial GCTs.
- Germinomas have a significantly better prognosis than nongerminomatous subtypes.
- Treatment strategies should be tailored based on histologic findings, with potential de-escalation of spinal axis radiation in select cases.
Abstract:
This 16-year retrospective study review sought to determine the factors influencing prognosis and treatment results of all patients with primary intracranial germ cell tumors treated at our hospital who were younger than 17 years of age at the time of diagnosis. A total of 44 patients were treated during the study period, including 32 males and 12 females with a male:female ratio of 2.67:1. The median age at diagnosis was 12 years and 5 months of age (range = 2-16 years). The 44 intracranial germ cell tumors consisted of 27 pure germinomas (61.4%) and 17 nongerminomatous germ cell tumors, including 10 mixed germ cell tumors (22.7%), three yolk sac tumors (7.8%), two immature teratomas (4.5%), and two choriocarcinomas (4.5%). Univariate analysis of prognostic factors using Kaplan-Meier survival estimates revealed that only histologic tumor type was correlated with outcome (P < 0.005). The projected 5-year overall survival and event-free survival rate of patients with germinomas vs those with intracranial germ cell tumors were 92.6%, 92.6% vs 47.3%, and 42.1%, respectively. Our analysis suggests that radiation involving the spinal axis has limited usefulness in patients with intracranial germ cell tumor, although better results have been obtained for germinomas using radiotherapy in this study.