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Clinical factors, management, and outcomes of children under 3 years old with central nervous system tumors: single-center experience

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Abstract

Purpose

Children under 3 years old represent a notable portion, about 25 to 30%, of all central nervous system tumor (CNS) cases. Their clinical course, prognosis, and treatment significantly differ from older children. This single-center retrospective study aims to comprehensively analyze survival factors in children under three diagnosed with CNS tumors.

Methods

Between April 2012 and December 2023, cases under 3 years of age with CNS tumors diagnosed at our center were retrospectively evaluated.

Results

Among 279 CNS tumor cases, 42 (15%) were evaluated. The 5-year overall and event-free survival rates were 67.4% (95% CI 47.5–81.1) and 39.8% (95% CI 24.2–55.0), respectively. Gender, symptom onset to diagnosis time, pathological neurological findings at diagnosis, and tumor location did not significantly impact survival (p > 0.05). However, cases with neurological symptoms showed significantly higher event-free survival rates (p < 0.05). Patients with embryonal tumors, metastases, inability for total surgical excision, relapsed/progressive diseases, and who under 1 year old had significantly lower survival rates (p < 0.05). Radiotherapy timing did not affect survival (p > 0.05). Event-free survival rates remained unchanged after the third year.

Conclusion

The current treatments have been observed to have a positive impact on survival rates. Nonetheless, there is a need for novel treatments for patients with embryonal tumors, metastases, aged under 1 year, and those where total surgical excision is not feasible or in cases with progressive/relapse disease. This study underscores the importance of the first 3 years regarding relapse, progression, or mortality risk.

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Data availability

Data access is available while maintaining patient confidentiality.

References

  1. Withrow DR, de Gonzalez AB, Lam CJ, Warren KE, Shiels MS (2019) Trends in pediatric central nervous system tumor incidence in the United States, 1998–2013. Cancer Epidemiol Biomark Prev 28:522–530

    Article  Google Scholar 

  2. Rickert CH, Paulus W (2001) Epidemiology of central nervous system tumors in childhood and adolescence based on the new WHO classification. Childs Nerv Syst 17:503–511

    Article  CAS  PubMed  Google Scholar 

  3. Peris-Bonet R, Martínez-García C, Lacour B, Petrovich S, Giner-Ripoll B, Navajans A et al (2006) Childhood central nervous system tumours–incidence and survival in Europe (1978–1997): report from Automated Childhood Cancer Information System project. Eur J Cancer 42:2064–2080

    Article  PubMed  Google Scholar 

  4. Duffner PK, Horowitz ME, Krischer JP, Burger PC, Cohen ME, Sanford RA et al (1999) The treatment of malignant brain tumors in infants and very young children: an update of the Pediatric Oncology Group experience. Neuro Oncol 1:152–161

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Varan A, Akalan N, Söylemezoğlu F, Zorlu F, Yalcın B, Akyüz C, Kutluk T, Büyükpamukcu M (2006) Central nervous system tumors in patients under three years of age: treatment results of a single institute. Pedatr Neurosurg 42:89–94

    Article  Google Scholar 

  6. European Rhabdoid Registry. European Rhabdoid Registry V5. http://www.rhabdoid.de/fileadmin/Daten/Pdfs/EU-RHAB_Protokoll_Stand_08.12.2016.pdf. Accessed 29 Nov 2016

  7. Lashford LS, Campbell RHA, Gattamaneni HR, Robinson K, Walker D, Bailey C (1996) An intensive multiagent chemotherapy regimen for brain tumors occurring in very young children. Arch Dis Child 74:219–223

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Stiller CA, Bunch KJ (1992) Brain and spinal tumors in children under 2 years: incidence and survival in Britain, 1975–1985. Br J Cancer 66(suppl 18):550–553

    Google Scholar 

  9. Rickert CH (1998) Epidemiological features of brain tumors in the first 3 years of life. Child’s Nerv Syst 14:547–550

    Article  CAS  Google Scholar 

  10. Reulecke BC, Erker CG, Fiedler BJ, Niederstadt T-U, Kurlemann G (2007) Brain tumors in children: initial symptoms and their influence on the time span between symptom onset and diagnosis. J Child Neurol 23:178–183

    Article  PubMed  Google Scholar 

  11. Young AL, Monuteaux MC, Cooney TM, Michelson KA (2023) Predictors of delayed diagnosis of pediatric CNS tumors in the emergency department. Pediatr Emerg Care 39(8):617–622

    Article  PubMed  Google Scholar 

  12. Hwang SW, Su JM, Jea A (2012) Diagnosis and management of brain and spinal cord tumors in the neonate. Semin Fetal Neonatal Med 17:202–206

    Article  PubMed  Google Scholar 

  13. Larouche V, Huang A, Bartels U, Bouffet E (2007) Tumors of the central nervous system in the first year of life. Pediatr Blood Cancer 49:1074–1082

    Article  PubMed  Google Scholar 

  14. Pollack IF, Agnihotri S, Broniscer A (2019) Childhood brain tumors: current management, biological insights, and future directions. J Neurosurg Pediatr 23:261–273

    Article  PubMed  PubMed Central  Google Scholar 

  15. Toescu SM, James G, Phipps K, Jeelani O, Thompson D, Hayward R, Aquilina K (2019) Intracranial neoplasms in the first year of life: results of a third cohort of patients from a single institution. Neurosurgery 84:636–646

    Article  PubMed  Google Scholar 

  16. Duffner PK, Horowitz ME, Krischer JP, Friedman HS, Burger PC, Cohen ME, Sanford RA, Mulhern RK, James HE, Freeman CR, Glen Seidel F, Kun (1993) Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 328:1725–1731

    Article  CAS  PubMed  Google Scholar 

  17. Rivera-Luna R, López E, Rivera-Marquez H, Rivera-Ortegón F, Altamirano-Alvarez E, Mercado G, Covarrubias G, Rueda-Franco F, Marhx-Bracho A, Gutiérrez P (2002) Survival of children under 3 years old with medulloblastoma: a study from the Mexican Cooperative Group for Childhood Malignancies (AMOHP). Childs Nerv Syst 18:38–42

    Article  PubMed  Google Scholar 

  18. Duffner PK, Cohen ME (1992) Changes in the approach to central nervous system tumors in childhood. Pediatr Clin North Am 39:859–877

    Article  CAS  PubMed  Google Scholar 

  19. Pötter R, Czech TH, Dieckmann K, Slavc I, Wimberger-Prayer D, Budka H (1998) Tumors of the central nervous system. In: Voûte PA, Kalifa C, Barrett A (eds) Cancer in Children: Clinical Management, 4th edn. Oxford University Press, Oxford, pp 170–192

    Google Scholar 

  20. Finlay JL, Boyett JM, Yates AJ, Wisoff JH, Milstein JM, Geyer JR, Bertolone SJ, McGuire P, Cherlow JM, Tefft M, Turski PA, Wara WM, Edwards M, Sutton LN, Berger MS, Epstein F, Ayers G, Allen JC (1995) Packer RJ for theChildren’s Cancer Group: Randomized phase III trial in childhood high-grade astrocytoma comparing vincristine, lomustine, and prednisone with the eight-drugs-in-1-day regimen. J Clin Oncol 13:112–123

    Article  CAS  PubMed  Google Scholar 

  21. Rivera-Luna R, Medina-Sanson A, Leal-Leal C, Pantoja-Guillen F, Zapata-Tarrés M, Cardenas-Cardos R, Barrera-Gómez R, Rueda-Franco F (2003) Brain tumors in children under 1 year of age: emphasis on the relationship of prognostic factors. Childs Nerv Syst 19:311–314

    Article  PubMed  Google Scholar 

  22. Fossey M, Li H, Afzal S, Carret A-S, Eisenstat DD, Fleming A, Hukin J, Hawkins C, Jabado N, Johnston D et al (2017) Atypical teratoid rhabdoid tumor in the first year of life: the Canadian ATRT registry experience and review of the literature. J Neuro-Oncol 132:155–162

    Article  CAS  Google Scholar 

  23. Grill J, Le Deley MC, Gambarelli D, Raquin MA, Couanet D, Pierre-Kahn A, Habrand JL, Doz F, Frappaz D, Gentet JC, Edan C, Chastagner P (2001) Kalifa C for the French Society of Pediatric Oncology: postoperative chemotherapy without irradiation for ependymoma in children under 5 years of age: a multicenter trial of the French Society of Pediatric Oncology. J Clin Oncol 19:1288–1296

    Article  CAS  PubMed  Google Scholar 

  24. Cohen BH, Geyer JR, Miller DC, Curran JG, Zhou T, Holmes E, Ingles SA, Dunkel IJ, Hilden J, Packer RJ, Pollack IF, Gajjar A, Finlay JL, Group CsO (2015) Pilot study of intensive chemotherapy with peripheral hematopoietic cell support for children less than 3 years of age with malignant brain tumors, the CCG-99703 phase I/II study. A report from the Children’s Oncology Group. Pediatr Neurol 53:31–46

    Article  Google Scholar 

  25. Espinoza JC, Haley K, Patel N, Dhall G, Gardner S, Allen J, Torkildson J, Cornelius A, Rassekh R, Bedros A, Etzl M, Garvin J, Pradhan K, Corbett R, Sullivan M, McGowage G, Stein D, Jasty R, Sands SA, Ji L, Sposto R, Finlay JL (2016) Outcome of young children with high-grade glioma treated with irradiation-avoiding intensive chemotherapy regimens: final report of the Head Start II and III trials. Pediatr Blood Cancer 63:1806–1813

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Dhall G, Grodman H, Ji L, Sands S, Gardner S, Dunkel IJ, McCowage GB, Diez B, Allen JC, Gopalan A, Cornelius AS, Termuhlen A, Abromowitch M, Sposto R, Finlay JL (2008) Outcome of children less than three years old at diagnosis with non-metastatic medulloblastoma treated with chemotherapy on the “Head Start” I and II protocols. Pediatr Blood Cancer 50:1169–1175

    Article  PubMed  Google Scholar 

  27. Geyer JR, Sposta R, Jennings M, Boyett JM, Axtell RA, Breiger D et al (2005) Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from the Children’s Cancer Group. J Clin Oncol 23:7621–7631

    Article  PubMed  Google Scholar 

  28. Dhall G, Neil O, SH, Ji L, Haley K, Whitaker AM, Nelson MD et al (2020) Excellent outcome of young children with noduler desmoplastic medulloblastoma treated on Head Start III: a multi-instutional, prospective clinical trial. Neuro Oncol 22:1862–1872

    Article  PubMed  PubMed Central  Google Scholar 

  29. Mazewski C, Kang G, Kellie S, Hayes L, Reddy A, Shaw D et al (2017) Treatment of young children with supratentorial PNET and high risk medulloblastoma without or with high dose methotrexate. A report from Children’s Oncology Group. SIOP 2017 abstracts. Pediatr Blood Cancer 64(suppl):O013

    Google Scholar 

  30. Von Bueren AO, Von Hoff K, Pietsch T, Gerber NU, Warmuth-Metz M, Deinlein F et al (2011) Treatment of young children with localized medulloblastoma by chemotherapy alone: results of the prospective, multicenter trial HIT 2000 confirming the prognostic impact of histology. Neuro Oncol 13(6):669–679

    Article  Google Scholar 

  31. Rutkowski S, Bode U, Deinlein F et al (2005) Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med 352:978–986

    Article  CAS  PubMed  Google Scholar 

  32. Metzger S, Welser A, Gerber NU, Otth M, Schelnemann K, Krayenbühl N et al (2022) Central nervous system tumors in children under 5 years of age: a report on treatment burden, survival and long-term outcomes. J Neurooncol 157:307–317

    Article  PubMed  PubMed Central  Google Scholar 

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Funding

The authors have no relevant financial information to disclose.

Author information

Authors and Affiliations

  1. Department of Pediatric Hematology Oncology, Marmara University, Marmara Faculty of Medicine, Fevzi Çakmak Mah, 34899, Pendik, Istanbul, Turkey

    Nurşah Eker & Gülnur Tokuç

  2. Republic of Turkey Ministry of Health, Cayırova District Health Directorate, Kocaeli, Turkey

    Alican Sarısaltık

  3. Department of Neurosurgery, Marmara University, Marmara Faculty of Medicine, Fevzi Çakmak Mah, 34899, Pendik, Istanbul, Turkey

    Adnan Dağçınar

  4. Department of Radiation Oncology, Marmara University, Marmara Faculty of Medicine, Fevzi Çakmak Mah, 34899, Pendik, Istanbul, Turkey

    Dilek Gül & Beste Melek Atasoy

  5. Department of Pediatric Hematology Oncology, Pendik Education and Research Hospital, Marmara University, Fevzi Çakmak Mah, 34899, Pendik, Istanbul, Turkey

    Barış Yılmaz & Burcu Tufan Taş

Authors
  1. Nurşah Eker
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  2. Gülnur Tokuç
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Contributions

Concept: N.E. Design: N.E and G.T. Data collection or processing: B.Y, B.T.T, D.G, B.A, and A.D. Analysis or interpretation: B.Y and A.S. Literature search: N.E and G.T. Writing: N.E, G.T, and A.S.

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Correspondence to Nurşah Eker.

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Eker, N., Tokuç, G., Sarısaltık, A. et al. Clinical factors, management, and outcomes of children under 3 years old with central nervous system tumors: single-center experience. Childs Nerv Syst (2024). https://doi.org/10.1007/s00381-024-06386-9

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