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Çocukluk Çağı Beyin Sapı Tümörleri

Year 2021, Volume: 9 Issue: 1, 18 - 22, 30.04.2021
https://doi.org/10.21765/pprjournal.907203

Abstract

Amaç: Bu çalışmanın amacı, beyin sapı tümör tanısı alan ve tedavi edilen hastalarımızın klinik bulguları, tedavi yaklaşımları ve sonuçlarını değerlendirmektir. Gereç ve Yöntem: Kliniğimizde, 2006 ile 2019 yılları arasında beyin sapı tümörü tanısı alan ve tedavi alan hastaların onkoloji dosyaları geriye yönelik olarak incelendi. Bulgular: Beyin sapı tümör tanısı almış 20 hasta çalışmaya dâhil edildi. Hastaların yaşı 2 ile 16 yıl arasında değişiyordu (median, 6,5 yıl). Cinsiyet dağılımı, 14’ü erkek (%70), 6’sı ise kızdı (%30). En sık başvuru şikâyetleri, yürüme bozukluğu (%60), baş ağrısı (%45) ve gözde kayma (%40) idi. En sık fizik muayene bulguları ise ataksi (%55) ve VI. Kranial sinir felci ( %55) idi. Tümör yerleşimleri, pons (%90), bulbus (%5) ve medulla (%5) idi. Dört hastaya parsiyel kitle eksizyonu yapılabilirken, bir hastada sadece biyopsi yapılabildi. Patolojik incelemede tanılar, grade II astrositom (n: 2), pilositik astrositom (n:1), primitif nöroektodermal tümör (n: 1) ve glioblastoma (n: 1) idi. En sık uygulanan tedavi yaklaşımı radyoterapi + kemoterapi (n: 7, %35) idi. İzlem süreleri 1 ay ile 11 yıl arasında değişiyordu (median, 10,5 ay). Genel yaşam oranı %19,5 idi. Sonuç: Çocukluk çağının birçok kanserinde ve hatta beyin tümörlerinde multidisipliner yaklaşımlarla yaşam oranlarında artışlar olmasına karşın, özellikle diffüz intrensek pons gliomlarında maalesef istenilen başarı elde edilememiştir. Özellikle kemoterapide yeni tedavi yaklaşımlara ihtiyaç vardır.

References

  • KAYNAKÇA 1. Jallo GI, Biser-Rohrbaugh A, Freed D. Brainstem gliomas. Childs Nerv Syst. 2004; 20(3): 143–53.
  • 2. Başarır M, Özek M. Beyin sapı tümörlerinde güncel tedavi seçenekleri. Türk Nöroşir Derg 2017; 27(1): 43-51.
  • 3. Robison NJ, Kieran MW. Diffuse intrinsic pontine glioma: a reassessment. J Neurooncol. 2014; 119(1): 7–15.
  • 4. Monje M, Mitra SS, Freret ME et al. Hedgehog-responsive candidate cell of origin for diffuse intrinsic pontine glioma. Proc Natl Acad Sci U S A. 2011 Mar 15;108(11):4453
  • 5. Fisher PG, Breiter SN, Carson BS, et al. A clinicopathologic reappraisal of brain stem tumor classification. Identification of pilocystic astrocytoma and fibrillary astrocytoma as distinct entities. Cancer. 2000 Oct 1;89(7):1569-76.
  • 6. Albright AL. Diffuse brainstem tumors: when is a biopsy necessary? Pediatr Neurosurg. 1996;24(5):252-5.
  • 7. El-Khouly FE, Veldhuijzen van Zanten SEM, Santa-Maria Lopez V, et al. Diagnostics and treatment of diffuse intrinsic pontine glioma: where do we stand?. J Neurooncol. 2019; 145(1):177–184.
  • 8. Veldhuijzen van Zanten SEM, Baugh J, Chaney B, et al. Development of the SIOPE DIPG network, registry and imaging repository: a collaborative effort to optimize research into a rare and lethal disease. J Neurooncol. 2017; 132(2): 255–266.
  • 9. Broniscer A, Laningham FH, Kocak M, et al. Intratumoral hemorrhage among children with newly diagnosed, diffuse brainstem glioma. Cancer. 2006 Mar 15;106(6):1364-71
  • 10. Epstein F, Constantini S. Practical decisions in the treatment of pediatric brain stem tumors. Pediatr Neurosurg. 1996;24(1):24-34.
  • 11. Schild SE, Stafford SL, Brown PD, et al. The results of radiotherapy for brainstem tumors. J Neurooncol. 1998 Nov;40(2):171-7.
  • 12. Jalali R, Raut N, Arora B, et al. Prospective evaluation of radiotherapy with concurrent and adjuvant temozolomide in children with newly diagnosed diffuse intrinsic pontine glioma. Int J Radiat Oncol Biol Phys. 2010; 77(1): 113–118.
  • 13. Kebudi R, Cakir FB, Agaoglu FY, et al. Pediatric diffuse intrinsic pontine glioma patients from a single center. Childs Nerv Syst. 2013; 29(4): 583–588.
  • 14. Hargrave D, Bartels U, Bouffet E. Diffuse brainstem glioma in children: critical review of clinical trials. Lancet Oncol. 2006 Mar;7(3):241-8.
  • 15. Kebudi R, Cakir FB. Management of diffuse pontine gliomas in children: recent developments. Paediatr Drugs. 2013; (5):351–362.

Brainstem Tumor in Children

Year 2021, Volume: 9 Issue: 1, 18 - 22, 30.04.2021
https://doi.org/10.21765/pprjournal.907203

Abstract

Objective: The aim of this study is to evaluate the clinical findings, treatment approaches and outcomes of our patients who were diagnosed and treated for brainstem tumor.
Material and Method: Between 2006 and 2019, oncology files of patients diagnosed with brain stem tumor and treated were analyzed retrospectively in our clinic.
Results: Twenty patients with brainstem tumor diagnosis were included in the study. The age of patients ranged from 2 to 16 years (median age, 6.5 years). The gender distribution was 14
males (70%) and 6 females (30%). The most common complaints were gait disturbance (60%), headache (45%), and strabismus (40%). The most common physical examination findings were ataxia (55%) and 6th cranial nerve palsy (55%). Tumor locations were pons (90%), bulbus (5%) and medulla (5%). Partial mass excision could be done in four patients, while only one
biopsy could be performed in one patient. In the pathological examination, the diagnoses were grade II astrocytoma (n: 2), pilocytic astrocytoma(n: 1) primitive neuroectodermal tumor (n:
1) and glioblastoma (n: 1). The most common treatment approach was radiotherapy + chemotherapy (n: 7, 35%). Follow-up time varied between 1 month and 11 years (median, 10.5 months). The overall survival rate was 19.5%.
Conclusion: Despite the increase in overall survival rates with multidisciplinary approaches in many cancers and even brain tumors of childhood, unfortunately the desired success could
not be achieved especially in diffuse intrinsic pons gliomas. New treatment approaches are needed, especially in chemotherapy.

References

  • KAYNAKÇA 1. Jallo GI, Biser-Rohrbaugh A, Freed D. Brainstem gliomas. Childs Nerv Syst. 2004; 20(3): 143–53.
  • 2. Başarır M, Özek M. Beyin sapı tümörlerinde güncel tedavi seçenekleri. Türk Nöroşir Derg 2017; 27(1): 43-51.
  • 3. Robison NJ, Kieran MW. Diffuse intrinsic pontine glioma: a reassessment. J Neurooncol. 2014; 119(1): 7–15.
  • 4. Monje M, Mitra SS, Freret ME et al. Hedgehog-responsive candidate cell of origin for diffuse intrinsic pontine glioma. Proc Natl Acad Sci U S A. 2011 Mar 15;108(11):4453
  • 5. Fisher PG, Breiter SN, Carson BS, et al. A clinicopathologic reappraisal of brain stem tumor classification. Identification of pilocystic astrocytoma and fibrillary astrocytoma as distinct entities. Cancer. 2000 Oct 1;89(7):1569-76.
  • 6. Albright AL. Diffuse brainstem tumors: when is a biopsy necessary? Pediatr Neurosurg. 1996;24(5):252-5.
  • 7. El-Khouly FE, Veldhuijzen van Zanten SEM, Santa-Maria Lopez V, et al. Diagnostics and treatment of diffuse intrinsic pontine glioma: where do we stand?. J Neurooncol. 2019; 145(1):177–184.
  • 8. Veldhuijzen van Zanten SEM, Baugh J, Chaney B, et al. Development of the SIOPE DIPG network, registry and imaging repository: a collaborative effort to optimize research into a rare and lethal disease. J Neurooncol. 2017; 132(2): 255–266.
  • 9. Broniscer A, Laningham FH, Kocak M, et al. Intratumoral hemorrhage among children with newly diagnosed, diffuse brainstem glioma. Cancer. 2006 Mar 15;106(6):1364-71
  • 10. Epstein F, Constantini S. Practical decisions in the treatment of pediatric brain stem tumors. Pediatr Neurosurg. 1996;24(1):24-34.
  • 11. Schild SE, Stafford SL, Brown PD, et al. The results of radiotherapy for brainstem tumors. J Neurooncol. 1998 Nov;40(2):171-7.
  • 12. Jalali R, Raut N, Arora B, et al. Prospective evaluation of radiotherapy with concurrent and adjuvant temozolomide in children with newly diagnosed diffuse intrinsic pontine glioma. Int J Radiat Oncol Biol Phys. 2010; 77(1): 113–118.
  • 13. Kebudi R, Cakir FB, Agaoglu FY, et al. Pediatric diffuse intrinsic pontine glioma patients from a single center. Childs Nerv Syst. 2013; 29(4): 583–588.
  • 14. Hargrave D, Bartels U, Bouffet E. Diffuse brainstem glioma in children: critical review of clinical trials. Lancet Oncol. 2006 Mar;7(3):241-8.
  • 15. Kebudi R, Cakir FB. Management of diffuse pontine gliomas in children: recent developments. Paediatr Drugs. 2013; (5):351–362.
There are 15 citations in total.

Details

Primary Language Turkish
Subjects Health Care Administration
Journal Section Original Articles
Authors

Buket Kara

Güler Yavaş

Yavuz Köksal

Publication Date April 30, 2021
Acceptance Date April 9, 2021
Published in Issue Year 2021 Volume: 9 Issue: 1

Cite

Vancouver Kara B, Yavaş G, Köksal Y. Çocukluk Çağı Beyin Sapı Tümörleri. pediatr pract res. 2021;9(1):18-22.