Abstract
Background
Congenital (< 3 months) and infant (3 to 11 months) brain tumors are biologically different from tumors in older children, but their epidemiology has not been studied comprehensively. Insight into epidemiological differences could help tailor treatment recommendations by age and increase overall survival (OS).
Methods
Population-based data from SEER were obtained for 14,493 0–19-year-olds diagnosed with CNS tumors 1990–2015. Congenital and infant age groups were compared to patients aged 1–19 years based on incidence, treatment, and survival using Chi-square and Kaplan–Meier analyses. Hazard ratios were estimated from univariate and multivariable Cox proportional hazards survival analyses.
Results
Between the < 3-month, 3–5-month, 6–11 month, and 1–19-year age groups, tumor type distribution differed significantly (p < 0.001). 5-year OS for all tumors was 36.7% (< 3 months), 56.0% (< 3–5 months), 63.8% (6–11 months), and 74.7% (1–19 years) (p < 0.001). Comparing between age groups by tumor type, OS was worst for < 3-month-olds with low-grade glioma, medulloblastoma, and other embryonal tumors; OS was worst for 3–5-month-olds with ependymoma, < 1-year-olds collectively with atypical teratoid-rhabdoid tumor, and 1–19-year-olds with high-grade glioma (HGG) (log rank p < 0.02 for all tumor types). Under 3-month-olds were least likely to receive any treatment for each tumor type and least likely to undergo surgery for all except HGG. Under 1-year-olds were far less likely than 1–19-year-olds to undergo both radiation and chemotherapy for embryonal tumors.
Conclusions
Subtype distribution, treatment patterns, and prognosis of congenital/infant CNS tumors differ from those in older children. Better, more standardized treatment guidelines may improve poorer outcomes seen in these youngest patients.
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Data availability
All data analyzed for this study is included in main tables, figures, and supplemental tables.
References
Fineberg R, Zahedi S, Eguchi M, Hart M, Cockburn M, Green AL (2020) Population-based analysis of demographic and socioeconomic disparities in pediatric CNS cancer survival in the United States. Sci Rep 10(1):4588
Ostrom QT, Patil N, Cioffi G, Waite K, Kruchko C, Barnholtz-Sloan JS (2020) CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2013–2017. Neuro Oncol. https://doi.org/10.1093/neuonc/noaa200
Lam S, Lin Y, Zinn P, Su J, Pan IW (2018) Patient and treatment factors associated with survival among pediatric glioblastoma patients: a surveillance, epidemiology, and end results study. J Clin Neurosci 47:285–293
Green AL, Furutani E, Ribeiro KB, Rodriguez GC (2017) Death within 1 month of diagnosis in childhood cancer: an analysis of risk factors and scope of the problem. J Clin Oncol 35(12):1320–1327
Wells EM, Packer RJ (2015) Pediatric brain tumors. Continuum (Minneap Minn) 21:373–396
Surveillance, Epidemiology, and End Results (SEER) Program. Number of Persons by Race and Hispanic Ethnicity for SEER Participants (2010 CensusData). https://seer.cancer.gov/registries/data.html; ICCC groups. https://seer.cancer.gov/iccc/iccc-who2008.html. Accessed 7 May 2020
Ostrom QT, de Blank PM, Kruchko C et al (2015) Alex’s Lemonade Stand Foundation infant and childhood primary brain and central nervous system tumors diagnosed in the United States in 2007–2011. Neuro Oncol 16(Suppl_10):x1–x36
Dressler EV, Dolecek TA, Liu M, Villano JL (2017) Demographics, patterns of care, and survival in pediatric medulloblastoma. J Neurooncol 132(3):497–506
Siegel DA, Li J, Ding H, Singh SD, King JB, Pollack LA (2019) Racial and ethnic differences in survival of pediatric patients with brain and central nervous system cancer in the United States. Pediatr Blood Cancer 66(2):e27501
Merchant TE, Pollack IF, Loeffler JS (2010) Brain tumors across the age spectrum: biology, therapy, and late effects. Semin Radiat Oncol 20(1):58–66
Colton MD, Goulding D, Beltrami A et al (2019) A U.S. population-based study of insurance disparities in cancer survival among adolescents and young adults. Cancer Med 8(10):4867–4874
Austin MT, Hamilton E, Zebda D et al (2016) Health disparities and impact on outcomes in children with primary central nervous system solid tumors. J Neurosurg Pediatr 18(5):585–593
Hicks D, Rafiee G, Schwalbe EC et al (2021) The molecular landscape and associated clinical experience in infant medulloblastoma: prognostic significance of second-generation subtypes. Neuropathol Appl Neurobiol 47(2):236–250
Junger ST, Andreiuolo F, Mynarek M et al (2020) Ependymomas in infancy: underlying genetic alterations, histological features, and clinical outcome. Childs Nerv Syst 36(11):2693–2700
Clarke M, Mackay A, Ismer B et al (2020) Infant high-grade gliomas comprise multiple subgroups characterized by novel targetable gene fusions and favorable outcomes. Cancer Discov 10(7):942–963
El-Ayadi M, Ansari M, Sturm D et al (2017) High-grade glioma in very young children: a rare and particular patient population. Oncotarget 8(38):64564–64578
Johann PD, Erkek S, Zapatka M et al (2016) Atypical teratoid/rhabdoid tumors are comprised of three epigenetic subgroups with distinct enhancer landscapes. Cancer Cell 29(3):379–393
Fruhwald MC, Hasselblatt M, Nemes K et al (2020) Age and DNA methylation subgroup as potential independent risk factors for treatment stratification in children with atypical teratoid/rhabdoid tumors. Neuro Oncol 22(7):1006–1017
Reddy AT, Strother DR, Judkins AR et al (2020) Efficacy of high-dose chemotherapy and three-dimensional conformal radiation for atypical teratoid/rhaboid tumor: a report from the Children’s Oncology Group trial ACNS0333. J Clin Oncol 38(11):1175–1185
Leary SES, Kilburn L, Geyer JR et al (2021) Vorinostat and isotretinoin with chemotherapy in young children with embryonal brain tumors: a report from the pediatric brain tumor consortium (PBTC-026). Neuro Oncol. https://doi.org/10.1093/neuonc/noab293
Acknowledgements
This work was supported in part by the Population Health Shared Resource of the University of Colorado Cancer Center support Grant P30CA046934. ALG is supported by a career development award from NINDS (1K08 NS102532-01) and the Luke’s Army Pediatric Cancer Research Fund St. Baldrick’s Scholar Award. A version of this project was presented at the 2020 International Symposium on Pediatric Neuro-Oncology (ISPNO).
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ALG conceived and designed the study. MH and AB led data gathering. MH, AB, AAM, and ALG performed data analysis, with population data analysis expertise provided by AAM. AG assisted with neuropathological classification of tumor groups. MH led manuscript preparation, and all authors assisted with writing and approved the final version.
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Hart, M., Anderson-Mellies, A., Beltrami, A. et al. Population-based analysis of CNS tumor diagnoses, treatment, and survival in congenital and infant age groups. J Neurooncol 157, 333–344 (2022). https://doi.org/10.1007/s11060-022-03967-z
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DOI: https://doi.org/10.1007/s11060-022-03967-z