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Causes of death in pediatric neuro-oncology: the sickkids experience from 2000 to 2017
Samuele Renzi1, Orli Michaeli1, Vijay Ramaswamy1
1Division of Hematology/Oncology, the Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 2M9, Canada.
Insights
Pediatric central nervous system (CNS) tumors are a leading cause of cancer death in children. This study found disease progression, particularly from high-grade gliomas and diffuse intrinsic pontine glioma, is the primary cause of mortality in pediatric CNS tumor patients.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Cancer Epidemiology
Background:
- Central nervous system (CNS) tumors are the most common pediatric solid tumors and a leading cause of cancer-related death in high-income countries.
- Limited data exists on the specific causes of mortality in children diagnosed with CNS tumors.
- Understanding mortality patterns is crucial for improving outcomes in pediatric neuro-oncology.
Purpose of the Study:
- To investigate the causes and patterns of mortality in a large cohort of pediatric patients diagnosed with CNS tumors.
- To identify specific tumor subtypes associated with higher mortality rates.
- To analyze the time from diagnosis to death and the primary causes of death in this population.
Main Methods:
- Retrospective review of patient charts for individuals diagnosed with CNS tumors between 2000 and 2017 at a Canadian Tertiary Care Children's Hospital.
- Data collection included tumor diagnosis, location, patient demographics, age at diagnosis and death, and cause of death.
- Analysis of mortality rates across different tumor subtypes and identification of primary causes of death.
Main Results:
- A total of 1274 patients were included, with 306 (24%) deaths. Mortality varied significantly by tumor subtype, from 3.1% for low-grade glioma (LGG) to 97.8% for diffuse intrinsic pontine glioma (DIPG).
- High-grade gliomas (HGG) and DIPG, though less common diagnoses, accounted for 49.3% of all deaths.
- Disease progression was the cause of death in 94.8% of patients; treatment-related toxicity and secondary malignancies were less common causes.
Conclusions:
- Disease progression is the predominant cause of death in pediatric CNS tumors, underscoring the need for more effective treatments for aggressive subtypes.
- Treatment-related mortality was low in this cohort, suggesting current therapeutic strategies are relatively safe but may not be sufficiently effective for certain tumors.
- Further research should focus on developing novel therapeutic strategies to improve survival rates for pediatric patients with dismal prognoses, particularly for DIPG and HGG.
Purpose:
Primary benign and malignant central nervous system (CNS) tumors are the most frequent solid tumors in the pediatric age and represent the leading cause of death by cancer in children in high income countries. However, information regarding specific causes of death in this population is still limited. The objective of this work was to investigate mortality in a large cohort of children diagnosed at our institution.
Methods:
We identified patients consecutively diagnosed with CNS tumor and treated at a Tertiary Care Canadian Children's Hospital between January 2000 and December 2017. Patient charts were reviewed and different variables such as tumor diagnosis, location, gender, age at diagnosis, age at death and cause of death collected.
Results:
Of 1274 patients, 306 (24%) succumbed to their disease. Mortality rate varied significantly according to tumor subtype, ranging from 3.1% in low grade glioma (LGG) to 97.8% in diffuse intrinsic pontine glioma (DIPG). While high grade gliomas (HGG) and DIPG represented only 6.3 and 7.1% of total diagnoses respectively, together they accounted for 49.3% of total deaths (n = 151). Median time from diagnosis to death was 15 months (4 days to 15 years) and shortest for DIPG (11 months). Two hundred and ninety patients (94.8%) died as a result of the primary disease, 4 of treatment-related toxicity, two patients' deaths were unrelated to the primary disease (idiopathic encephalopathy and domestic fire) whereas 10 patients succumbed to a secondary malignancy. Of note, four of these ten patients had a confirmed underlying cancer predisposition syndrome.
Conclusion:
Disease progression is the main cause of death in children with brain tumor, while treatment related mortality is low in this series. Research should continue to focus on improving treatment strategies for patients whose prognosis remains dismal.
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