Causes of death in pediatric neuro-oncology: the sickkids experience from 2000 to 2017
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CLINICAL STUDY
Causes of death in pediatric neuro‑oncology: the sickkids experience from 2000 to 2017 Samuele Renzi1 · Orli Michaeli1 · Vijay Ramaswamy1 · Annie Huang1 · Derek Stephens2 · Bryan Maguire2 · Uri Tabori1 · Eric Bouffet1 · Ute Bartels1 Received: 1 June 2020 / Accepted: 6 August 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract 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. Keywords Brain tumors · Pediatric · Mortality · Central nervous system tumors · CNS
Introduction
Samuele Renzi and Orli Michaeli have contributed equally to this work. * Ute Bartels [email protected] 1
Division of Hematology/Oncology, the Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 2M9, Canada
Division of Statistics, the Hospital for Sick Children, Toronto, Canada
2
Primary central nervous system (CNS) tumors are the most common solid tumors in children, and they represent the second most common pediatric malignancies after leukemia, accounting for almost 25% of all childhood cancers in high income countries [1–3]. Yet, they are infrequent, with an estimated incidence which varies by country from 1.12 to 5.57 cases per 100,000 children each year [4]. Althoug
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