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Medulloblastoma in children: 84 patients from a single institution

Year 2020, Volume: 45 Issue: 1, 56 - 62, 31.03.2020
https://doi.org/10.17826/cumj.638237

Abstract

Purpose: Medulloblastoma is the most common malignant brain tumor in children. Current treatment strategy for medulloblastoma includes surgery, followed by radiotherapy and chemotherapy. In the present study, we analyze the clinical features, risk categories, treatment, and outcome of the patients with medulloblastoma treated in our institution in the last 15 years.
Materials and Methods: 84 patients with a diagnosis of medulloblastoma at our institution between January 2004 and December 2018 were included in the sudy. Hospital files of the patients were investigated and clinical and epidemiological characteristics, histopathology, treatment modalities, prognostic criteria and survival rates of patients were noted in data form.
Results: There were 45 males and 39 females with a median age of 8 years at the time of diagnosis. Approximately 12% of the patients were Syrian refugees. 54 patients were included in average-risk group and 30 in high-risk group based on the age at the time of diagnosis, extent of the residual tumor after surgery and Chang staging system. Five-year overall survival and event-free survival rates were 58.1% and 57.6% respectively. The prognostic factors having a statistically significant influence on survival were histopathological group, type of surgery and risk group. Only the risk group was found to have an impact on survival in multivariate analysis.
Conclusion: Risk group, utilizing the age of the patient, degree of surgical resection and metastatic disease, is the most important parameter having an impact on survival of patients with medulloblastoma. 

References

  • 1. Udaka YT, Packer RJ. Pediatric Brain Tumors. Neurol Clin. 2018;36:533-556.2. Bautista F, Fioravantti V, de Rojas T, Carceller F, Madero L, Lassaletta A, Moreno L. Medulloblastoma in children and adolescents: a systematic review of contemporary phase I and II clinical trials and biology update. Cancer Med. 2017;6:2606-2624.3. Ramaswamy V, Taylor MD. Medulloblastoma: From Myth to Molecular. J Clin Oncol. 2017;35:2355-2363.4. Ramaswamy V, Remke M, Bouffet E, Bailey S, Clifford SC, Doz F, et al. Risk stratification of childhood medulloblastoma in the molecular era: the current consensus. Acta Neuropathol. 2016;131:821-831.5. Dufour C, Beaugrand A, Pizer B, Micheli J, Aubelle MS, Fourcade A, et al. Metastatic Medulloblastoma in Childhood: Chang's Classification Revisited. Int J Surg Oncol. 2012;2012:245385.6. Pickles JC, Hawkins C, Pietsch T, Jacques TS. CNS embryonal tumours: WHO 2016 and beyond. Neuropathol Appl Neurobiol. 2018;44:151-162.7. Lassaletta A. Medulloblastoma in infants: the never-ending challenge. Lancet Oncol. 2018;19:720-721. 8. Gupta T, Shirsat N, Jalali R. Molecular Subgrouping of Medulloblastoma: Impact Upon Research and Clinical Practice. Curr Pediatr Rev. 2015;11:106-119.9. Goschzik T, Schwalbe EC, Hicks D, Smith A, Zur Muehlen A, Figarella-Branger D, et al. Prognostic effect of whole chromosomal aberration signatures in standard-risk, non-WNT/non-SHH medulloblastoma: a retrospective, molecular analysis of the HIT-SIOP PNET 4 trial. Lancet Oncol. 2018;19:1602-1616.10. Robinson GW, Rudneva VA, Buchhalter I, Billups CA, Waszak SM, Smith KS, et al. Risk-adapted therapy for young children with medulloblastoma (SJYC07): therapeutic and molecular outcomes from a multicentre, phase 2 trial. Lancet Oncol. 2018;19:768-784.11. Kool M, Korshunov A, Remke M, Jones DT, Schlanstein M, Northcott PA, et al. Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomas. Acta Neuropathol. 2012;123:473-484. 12. Phi JH, Lee J, Wang KC, Cho BK, Kim IO, Park CK, et al. Cerebrospinal fluid M staging for medulloblastoma: reappraisal of Chang's M staging based on the CSF flow. Neuro Oncol. 2011;13:334-344.13. Robinson GW, Rudneva VA, Buchhalter I, Billups CA, Waszak SM, Smith KS, et al. Risk-adapted therapy for young children with medulloblastoma (SJYC07): therapeutic and molecular outcomes from a multicentre, phase 2 trial. Lancet Oncol. 2018;19:768-784.14. Ertan Y, Sezak M, Demirağ B, Kantar M, Cetingül N, Turhan T, et al. Medulloblastoma: clinicopathologic evaluation of 42 pediatric cases. Childs Nerv Syst. 2009;25:353-356.15. Akyüz C, Varan A, Küpeli S, Akalan N, Söylemezoglu F, Zorlu F, et al. Medulloblastoma in children: a 32-year experience from a single institution. J Neurooncol. 2008;90:99-103.16. Mehrvar A, Faranoush M, Hedayati Asl AA, Tashvighi M, Fazeli MA, Qaddoumi I, Mehrvar N, Sobuti B, Jafarpour A, Ravan Parsa R, Zangooei R, Alebouyeh M, Vossough P. Childhood central nervous system tumors at MAHAK's Pediatric Cancer Treatment and Research Center (MPCTRC), Tehran, Iran. Childs Nerv Syst. 2014;30:491-496.17. Pinho RS, Andreoni S, Silva NS, Cappellano AM, Masruha MR, Cavalheiro S, Vilanova LC. Pediatric central nervous system tumors: a single-center experience from 1989 to 2009. J Pediatr Hematol Oncol. 2011;33:605-609.18. Küpeli S, Yalçın B, Bilginer B, Akalan N, Haksal P, Büyükpamukçu M. Posterior fossa syndrome after posterior fossa surgery in children with brain tumors. Pediatr Blood Cancer. 2011;56:206-210.19. Albright AL, Wisoff JH, Zeltzer P, Boyett J, Rorke LB, Stanley P, Geyer JR, Milstein JM. Prognostic factors in children with supratentorial (nonpineal) primitive neuroectodermal tumors. A neurosurgical perspective from the Children's Cancer Group. Pediatr Neurosurg. 1995;22:1-7.20. Kupeli S, Bicakci K, Sezgin G, Bayram I. Evaluation of late cerebral vascular complications in cranially irradiated pediatric cancer patients with magnetic resonance angiography. Tumori. 2018;104:381-387.21. Tarbell NJ, Friedman H, Polkinghorn WR, Yock T, Zhou T, Chen Z, Burger P, Barnes P, Kun L. High-risk medulloblastoma: a pediatric oncology group randomized trial of chemotherapy before or after radiation therapy (POG 9031). J Clin Oncol. 2013;31:2936-2941.22. Kebudi R, Bayram I, Yagci-Kupeli B, Kupeli S, Sezgin G, Pekpak E, et al. Refugee children with cancer in Turkey. Lancet Oncol. 2016;17:865-867.

Çocukluk çağında medulloblastom: Tek merkezden 84 hasta

Year 2020, Volume: 45 Issue: 1, 56 - 62, 31.03.2020
https://doi.org/10.17826/cumj.638237

Abstract

Amaç: Medulloblastom, çocuklarda en sık görülen malign beyin tümörüdür. Medulloblastom için mevcut tedavi stratejisi cerrahi, ardından radyoterapi ve kemoterapiyi içerir. Bu çalışmada kurumumuzda son 15 yılda tedavi edilen medulloblastomlu hastaların klinik özellikleri, risk kategorileri, tedavileri ve sonuçlar analiz edilmiştir.
Gereç ve Yöntem: Çalışmamıza Ocak 2004 - Aralık 2018 tarihleri arasında medulloblastom tanısı alan 84 hasta dahil edildi. Hastaların hastane dosyaları incelendi ve klinik ve epidemiyolojik özellikler, histopatoloji, tedavi yöntemleri, prognostik kriterler ve sağkalım oranları veri formunda not edildi.
Bulgular: Tanı anında ortanca yaşı 8 olan 45 erkek ve 39 kadın vardı. Hastaların yaklaşık % 12'si Suriyeli mültecilerdi. Tanı sırasındaki yaşa, cerrahi sonrası rezidüel tümörün boyutuna ve Chang evreleme sistemine bağlı olarak standart risk grubuna 54, yüksek risk grubuna 30 hasta dahil edildi. Beş yıllık genel sağkalım ve olaysız sağkalım oranları sırasıyla% 58.1 ve% 57.6 idi. Sağkalım üzerinde istatistiksel olarak anlamlı bir etkiye sahip prognostik faktörler histopatolojik grup, cerrahi tip ve risk grubuydu. Çok değişkenli analizde, sadece risk grubunun sağkalımı etkilediği bulundu.
Sonuç: Hastanın yaşını, cerrahi rezeksiyon derecesini ve metastaz durumunu kullanan risk grubu parametresi medulloblastomlu hastaların sağkalımına etki eden en önemli faktördür.

References

  • 1. Udaka YT, Packer RJ. Pediatric Brain Tumors. Neurol Clin. 2018;36:533-556.2. Bautista F, Fioravantti V, de Rojas T, Carceller F, Madero L, Lassaletta A, Moreno L. Medulloblastoma in children and adolescents: a systematic review of contemporary phase I and II clinical trials and biology update. Cancer Med. 2017;6:2606-2624.3. Ramaswamy V, Taylor MD. Medulloblastoma: From Myth to Molecular. J Clin Oncol. 2017;35:2355-2363.4. Ramaswamy V, Remke M, Bouffet E, Bailey S, Clifford SC, Doz F, et al. Risk stratification of childhood medulloblastoma in the molecular era: the current consensus. Acta Neuropathol. 2016;131:821-831.5. Dufour C, Beaugrand A, Pizer B, Micheli J, Aubelle MS, Fourcade A, et al. Metastatic Medulloblastoma in Childhood: Chang's Classification Revisited. Int J Surg Oncol. 2012;2012:245385.6. Pickles JC, Hawkins C, Pietsch T, Jacques TS. CNS embryonal tumours: WHO 2016 and beyond. Neuropathol Appl Neurobiol. 2018;44:151-162.7. Lassaletta A. Medulloblastoma in infants: the never-ending challenge. Lancet Oncol. 2018;19:720-721. 8. Gupta T, Shirsat N, Jalali R. Molecular Subgrouping of Medulloblastoma: Impact Upon Research and Clinical Practice. Curr Pediatr Rev. 2015;11:106-119.9. Goschzik T, Schwalbe EC, Hicks D, Smith A, Zur Muehlen A, Figarella-Branger D, et al. Prognostic effect of whole chromosomal aberration signatures in standard-risk, non-WNT/non-SHH medulloblastoma: a retrospective, molecular analysis of the HIT-SIOP PNET 4 trial. Lancet Oncol. 2018;19:1602-1616.10. Robinson GW, Rudneva VA, Buchhalter I, Billups CA, Waszak SM, Smith KS, et al. Risk-adapted therapy for young children with medulloblastoma (SJYC07): therapeutic and molecular outcomes from a multicentre, phase 2 trial. Lancet Oncol. 2018;19:768-784.11. Kool M, Korshunov A, Remke M, Jones DT, Schlanstein M, Northcott PA, et al. Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomas. Acta Neuropathol. 2012;123:473-484. 12. Phi JH, Lee J, Wang KC, Cho BK, Kim IO, Park CK, et al. Cerebrospinal fluid M staging for medulloblastoma: reappraisal of Chang's M staging based on the CSF flow. Neuro Oncol. 2011;13:334-344.13. Robinson GW, Rudneva VA, Buchhalter I, Billups CA, Waszak SM, Smith KS, et al. Risk-adapted therapy for young children with medulloblastoma (SJYC07): therapeutic and molecular outcomes from a multicentre, phase 2 trial. Lancet Oncol. 2018;19:768-784.14. Ertan Y, Sezak M, Demirağ B, Kantar M, Cetingül N, Turhan T, et al. Medulloblastoma: clinicopathologic evaluation of 42 pediatric cases. Childs Nerv Syst. 2009;25:353-356.15. Akyüz C, Varan A, Küpeli S, Akalan N, Söylemezoglu F, Zorlu F, et al. Medulloblastoma in children: a 32-year experience from a single institution. J Neurooncol. 2008;90:99-103.16. Mehrvar A, Faranoush M, Hedayati Asl AA, Tashvighi M, Fazeli MA, Qaddoumi I, Mehrvar N, Sobuti B, Jafarpour A, Ravan Parsa R, Zangooei R, Alebouyeh M, Vossough P. Childhood central nervous system tumors at MAHAK's Pediatric Cancer Treatment and Research Center (MPCTRC), Tehran, Iran. Childs Nerv Syst. 2014;30:491-496.17. Pinho RS, Andreoni S, Silva NS, Cappellano AM, Masruha MR, Cavalheiro S, Vilanova LC. Pediatric central nervous system tumors: a single-center experience from 1989 to 2009. J Pediatr Hematol Oncol. 2011;33:605-609.18. Küpeli S, Yalçın B, Bilginer B, Akalan N, Haksal P, Büyükpamukçu M. Posterior fossa syndrome after posterior fossa surgery in children with brain tumors. Pediatr Blood Cancer. 2011;56:206-210.19. Albright AL, Wisoff JH, Zeltzer P, Boyett J, Rorke LB, Stanley P, Geyer JR, Milstein JM. Prognostic factors in children with supratentorial (nonpineal) primitive neuroectodermal tumors. A neurosurgical perspective from the Children's Cancer Group. Pediatr Neurosurg. 1995;22:1-7.20. Kupeli S, Bicakci K, Sezgin G, Bayram I. Evaluation of late cerebral vascular complications in cranially irradiated pediatric cancer patients with magnetic resonance angiography. Tumori. 2018;104:381-387.21. Tarbell NJ, Friedman H, Polkinghorn WR, Yock T, Zhou T, Chen Z, Burger P, Barnes P, Kun L. High-risk medulloblastoma: a pediatric oncology group randomized trial of chemotherapy before or after radiation therapy (POG 9031). J Clin Oncol. 2013;31:2936-2941.22. Kebudi R, Bayram I, Yagci-Kupeli B, Kupeli S, Sezgin G, Pekpak E, et al. Refugee children with cancer in Turkey. Lancet Oncol. 2016;17:865-867.
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Details

Primary Language English
Subjects Paediatrics
Journal Section Research
Authors

Serhan Küpeli 0000-0001-7271-1803

Gülay Sezgin This is me

İbrahim Bayram This is me

Atila Tanyeli This is me

Publication Date March 31, 2020
Acceptance Date December 4, 2019
Published in Issue Year 2020 Volume: 45 Issue: 1

Cite

MLA Küpeli, Serhan et al. “Medulloblastoma in Children: 84 Patients from a Single Institution”. Cukurova Medical Journal, vol. 45, no. 1, 2020, pp. 56-62, doi:10.17826/cumj.638237.