05Sep 2019

CURRENT MANAGEMENT OF BRAINSTEM GLIOMAS.

  • Department of Radiation Oncology, Bahcesehir University,Goztepe, Istanbul/Turkey.
  • Clinics of Radiation Oncology, Mersin City Hospital, Mersin, Turkey.
  • Department of Medical Oncology, Baskent University Medical Faculty, Adana, Turkey .
  • Baskent University Medical Faculty, Department of Radiation Oncology, 01120, Adana, Turkey.
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Brainstem gliomas (BSGs) which have extremely poor prognosis represent up to 20% of all pediatric brain tumors, while only for 1-2% of adult brain tumors. Because of high mortality and morbidity rates of biopsy or any surgical excision, treatment decisions are usually based on MRI findings alone and not include a histopathological diagnosis. However, technologic advances in recent years made it possible to surgically approach BSGs with reduced mortality and morbidity, and therefore, surgery became a more commonly practiced treatment modality than before in such patients. Accessible evidence suggest the conventionally fractionated total dose of 54-60 Gy (1.8-2.0 Gy per fraction) RT as the standard of care for BSG patients, however, it should be noted that treatment response is almost always transient and followed by inevitable fatal recurrences. For this reason, studies focusing on molecules that may enhance the effectiveness of RT may potentially play an important role in changing the poor fate of BSG patients, but, this can only be achievable by cooperative studies targeting tumor biology to determine the genetic characteristics and molecular markers of BSG.

  1. Smith MA, Freidlin B, Ries LA, et al. Trends in reported incidence of primary malignant brain tumors in children in the United States. J Natl Cancer Inst 1998; 90:1269-77.
  2. Adult brainstem gliomas. Reyes-Botero G, Mokhtari K, Martin-Duverneuil N, Delattre JY, Laigle-Donadey F. Oncologist. 2012;17:388-97.
  3. Kansal S, Jindal A, Mahapatra AK. Brain stem glioma: A study of 111 patients. Indian J Cancer 1999; 36:99-108.
  4. Selvapandian S, Rajshekhar V, Chandy MJ. Brainstem glioma: comparative study of clinico-radiological presentation, pathology and outcome in children and adults. ActaNeurochir (Wein) 1999; 141:721-.27.
  5. Freeman CR, Farmer J-P. Pediatric brain stem gliomas: a review. Int J RadiatOncolBiol Phys 1998; 40:265-71.
  6. Packer RJ, Allen J, Nielsen S, et al. Brainstem glioma: Clinical manifestations of meningeal gliomatosis. Ann Neurol 1983; 14:177-82.
  7. Silbergeld D, Berger M, Griffin B, et al. Brainstem glioma with multiple intraspinal metastases during life: Case report and review of the literature. PediatrNeurosci 1988; 14:103-7.
  8. Fisher PG, Breiter SN, Carson BS, et al. A clinicopathologic reappraisal of brain stem tumor classification: Identification of pilocytic astrocytoma and fibrillary astrocytoma as distinct entities. Cancer 2000; 89:1569-76.
  9. Khatib ZA, Heideman RL, Kovnar EH, et al. Predominance of pilocytic histology in dorsally exophytic brain stem tumors. PediatrNeurosurg 1994; 20:2-10.
  10. Lonser RR, Glenn GM, Walther M, et al. Von Hippel-Lindau disease. Lancet 2003;361(9374):2059-67.
  11. Monje M, Mitra S, Freret M, et al. Hedhehog-responsive candidate cell of origin for diffuse intrinsic pontine glioma. Proc Natl AcadSci USA, 1986; 108(11):4453-8.
  12. Cheng Y, Ng H, Zhang S, et al. Genetic alterations in pediatric high-grade astrocytoma. Hum Pathol. 1999;30:1284-90.
  13. Gillbertson R, Hill D, Hernan R, et al. ERBB1 is amplified and overexpressed in high-grade diffusely infiltrative pediatric brainstem glioma. Clin Cancer Res 2003;9:3620-4.
  14. Zarghoni M, Bartels U, Lee E, et al. Whole-genome profiling of pediatric diffuse intrinsic pontine gliomas highlights platelet-derived growth factor receptor α and poly (ADP-ribose) polymerase as potential therapeutic targets. J ClinOncol 2010; 28:1337-.44.
  15. Warren K, Killian K, Suuriniemi M, et al. Genomic aberrations in pediatric diffuse intrinsic pontine gliomas. Neuro Oncol 2012;14(3):326-32
  16. Kinkingereder P, Willeit P, Simon T, et al. Diagnostic value and safety of stereotactic biopsy for brainstem tumors: a systematic review and meta-analysis of 1480 cases. Neurosurgery 2013;72(6):873-81.
  17. Paugh B, Broniscer A, Qu C, et al. Genome ?wide analyses identify recurrent amplifications of receptor tyrosine kinases and cell-cycle regulatory genes in diffuse intrinsc pontine glioma. J ClinOncol 2010;29:3999-4006.
  18. Bartels U, Hawkins C, Gilbert V, et al. Proceedings of the diffuse intrinsic pontine glioma (DIPG) Toronto Think Tank: advancing basic and translational research and cooperation in DIPG. J Neurooncol 2011;105:119-125.
  19. Khuong-Quang DA, Buczkowicz P, Rakopoulos P, et al. K27M mutation in histone H3.3 defines clinically and biologically distinct subgroups of pediatric diffuse intrinsic pontine gliomas. ActaNeuropathol 2012;124(3):439-47.
  20. Thon N, Tonn JC, Kreth FW. The surgical perspective in precision treatment of diffuse gliomas.Onco Targets Ther. 2019;12:1497-508.
  21. Donaldson SS, Laningham F, Fisher PG. Advances toward an understanding of brainstem gliomas. J ClinOncol 2006;24:1266-72.
  22. Tzika AA, Astrakas LG, Zarifi MK, et al. Spectroscopic and perfusion magnetic resonance imaging predictors of progression in pediatric brain tumors. Cancer 2004;100:1246-56.
  23. Gauvain KM, McKinstry RC, Mukherjee P, et al. Evaluating pediatric brain tumor cellularity with diffusion-tensor imaging. Am J Roentgenol 2001;177:449-454
  24. Tien RD, Felsberg GJ, Friedman H, et al. MR imaging of high-grade cerebral gliomas: Value of diffusion-weighted echoplanar pulse sequences. Am J Roentgenol 1994;162:671-7.
  25. Guo AC, Cummings TJ, Dash RC, et al. Lymphomas and high-grade astrocytomas: Comparison of water diffusibility and histologic characteristics. Radiology 2002;224:177-83.
  26. Taylor JS, Ogg RJ, Langston JW. Proton MRS of pediatric brain tumors, in Castillo M (ed): Neuroimaging Clinics of North America. Philadelphia, PA, WB Saunders 1998, pp 753-79.
  27. Broniscer A, Gajjar A, Bhargava R, et al. Brainstem involvement in children with neurofibromatosis type 1: Role of magnetic resonance imaging and spectroscopy in the distinction from diffuse pontine glioma. Neurosurgery 1997; 40:331-8.
  28. Sevick RJ, Barkowich AJ, Edwards MS, et al. Evoluation of white matter lesions in neurofibromatosis type 1: MR findings. Am J Roentgenol 1992;159:171-5.
  29. Albright AL, Guthkelch AN, Packer RJ, et al. Prognostic factors in pediatric brain-stem gliomas. Journal of Neurosurgery 1986;65:751-5.
  30. Freeman CR, Krischer JP, Sanford RA, et al. Final results of a study of escalating doses of hyperfractionated radiotherapy in brain stem tumors in children: a Pediatric Oncology Group study. Int J RadiatOncolBiol Phys 1993; 27:197-206.
  31. Broniscer A, Laningham FH, Sanders RP, et al. Young age may predict a better outcome for children with diffuse pontine glioma. Cancer 2008;113:566-72.
  32. Panitch HS, Berg BO. Brain stem tumors of childhood and adolescence. American Journal of Diseases of Children 1970;119:465-72.
  33. Molloy PT, Bilaniuk LT, Vaughan SN, et al. Brainstem tumors in patients with neurofibromatosis type 1: a distinct clinical entity. Neurology 1995;10:1897-902.
  34. Rachinger W, Grau SJ, Holtmannsp?tter M, Herms J, et al. Serial strerotactic biopsy of brainstem lesions in adults improves diagnostic accuracy compared with MRI only. J NeourolNeurosurgPsyhiatry 2009;80: 1134-9.
  35. Dellaretti M, Touzet G, Reyns N, et al. Correlation among magnetic resonance imaging findings, prognostic factors for survival, and histological diagnosis of intrinsic brainstem lesions in children. J Neurosurg Pediatrics 2011;8:539-43.
  36. Sufit A, Donson AM, Birks DK, et al. Diffuse intrinsic pontine tumors:a study of primitive neuroectodermal tumors versus the more common difffuse intrinsic pontine gliomas. J NeurosurgPediatr 2012;10:81-8.
  37. Ogiwara H, Morota N. The efficacy of a biopsy of intrinsic brainstem lesions for decisions making of the treatments. Childs NervSyst 2013;29:833-7.
  38. Del Brutto, Mosquera A. Brainstem tuberculoma mimicking glioma: the role of antituberculous drugs as a diagnostic tool. Neurology 1999;52:210-1.
  39. Weaver KD, Armao D, Wiley JM, et al. Histiocytic lesion mimicking intrinsic brainstem neoplasm. J Neurosurg 1999;91:1037-40.
  40. Oka K, Kin Y, Go Y, et al. Neuroendoscopic approach to tectal tumors: a consecutive series. J Neurosurg 1999,91:964-70.
  41. Wolburg H, Noell S, Wolburg-Buchholz K, et al. Agrin, aquaprin-4 and astrocyte polarity as an important feature of the blood: brain barrier. Neuroscientist 2009;15,180-93.
  42. Cohen KJ, Heideman RL, Zhou T, et al. Temozolomide in the treatment of children with newly diagnosed diffuse intrinsic pontine gliomas: a report from the Children's Oncology Group. Neuro Oncol1 2011;3:410-6.
  43. 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 RadiatOncol Phys 2009;77(1):113-8.
  44. Sharp JR, Bouffet E, Stempak D, et al. A multi-centre Canadian pilot study of metronomic temozolomide combined with radiotherapy for newly diagnosed pediatric brainstem glioma. Eur J Cancer 2010;46(18):3271-9.
  45. Sanghavi S, Needle M, Krailo M, et al. A phase I study of topotecan as a radiosensitizer for brainstem glioma of childhood: first report of the Children?s Cancer Group-0952. Neuro Oncol 2003;5:8-13.
  46. Bernier-Chastagner V, Grill J, Doz F, et al. Topotecan as a radiosensitizer in the treatment of children with malignant diffuse brainnstem gliomas: results of a French Society of Pediatric Oncology Phase II study. Cancer 2005;104:2792-7.
  47. Aquino-Parsons C, Hukin J, Green A. Concurrent carbogen and radiation therapy in children with high-risk brainstem gliomas. Pediatr Blood Cancer 2008;50(2):397-9.
  48. Bradley KA, Zhou T, McNall-Knapp RY, et al. Motexafin gadolinium and involved field radiation therapy for intrinsic pontine glioma of childhood: a children?s Oncology Group phase II study. Int J RadiatOncol Phys 2013;85(1):e55-60.
  49. Marcus K, Dutton S, Barnes P, et al. A phase I trial of etanidazole and hyperfractionated radiotherapy in children with with diffuse brainstem glioma. Int J Radaiat. Oncol. Biol. Phys 2003;55:1182-5.
  50. Wakabayashi T, Yoshida J, Mizuno M, et al. Effectiveness of interferon-beta, ACNU, and radiation therapy in pediatric patients with brainstem glioma. Neurologica Medico-Chirurgica 1992;32:942-6.
  51. Packer RJ, Prados M, Phillips P, et al. Treatment of children with newly diagnosed brain stem gliomas with intravenous recombinant beta interferon and hyperfractionated radiation therapy: a childrens cancer group phase I/II study. Cancer 1996;77:2150-6.
  52. Hargrave D, Bartels U, Bouffet E. Diffuse brainstem glioma in children: critical rewiev of clinical trials. Lancet Oncol 2006;7(3):241-8.
  53. Freeman CR, Suissa S. Brain stem tumours in children: Results of a survey of 62 patients treated with radiotherapy. Int J RadiatOncolBiol Phys 1986;12:1823-8.
  54. Guiney MJ, Smith JG, Hughes P, et al. Contemporary management of adult and pediatric brain stem gliomas. Int J RadiatOncolBiol Phys 1993;25:235-41.
  55. Freeman CR, Krischer J, Sanford RA, et al. Hyperfractionated radiotherapy in brain stem tumors: Results of a Pediatric Oncology Group Study. Int J RadiatOncolBiol Phys 1988; 15:311-8.
  56. Freeman CR, Krischer J, Sanford RA, et al: Hyperfractionated radiotherapy for brain stem tumors:Results at the 7020 cGy dose level of Pediatric Oncology Group Study 8495. Cancer 1991;68:474-81.
  57. Walter AW, Gajjar A, Ochs JS, et al. Carboplatin and etoposide with hyperfractionated radiotherapy in children with newly diagnosed diffuse pontine gliomas: A phase I/II study. Med PediatrOncol 1998;30:28-33.
  58. Mandell LR, Kadota R, Freeman C, et al. There is no role for hyperfractionated radiotherapy in the management of children with newly diagnosed diffuse intrinsic brainstem tumors: Results of a Pediatric Oncology Group Phase III Trial comparing conventional vs. hyperfractionated radiotherapy. Int J RadiatOncolBiol Phys 1999;43:959-64.
  59. Freeman CR, Kepner J, Kun LE, et al. A detrimental effect of a combined chemotherapyradiotherapy approach in children with diffuse intrinsic brain stem gliomas. Int J RadiatOncolBiol Phys 2000;47:561-4.
  60. Packer RJ, Littman PA, Sposto RM, et al. Results of a pilot study of hyperfractionated radiation therapy for children with brain stem gliomas. Int J RadiatOncolBiol Phys 1987;13:1647-51.
  61. Packer RJ, Allen JC, Goldwein JL, et al. Hyperfractionated radiotherapy for children with brainstem gliomas: A pilot study using 7,200 cGy. Ann Neurol 1990;27:167-173
  62. Packer RJ, Boyett JM, Zimmerman RA, et al: Outcome of children with brain stem gliomas after treatment with 7800 cGy of hyperfractionated radiotherapy. Cancer 1994;74:1827-34.
  63. Packer RJ, Boyett JM, Zimmerman RA, et al. Hyperfractionated radiotherapy (72 Gy) for children with brain stem gliomas: A Children?s Cancer Group phase I/II trial. Cancer 1993;72:1414-.21.
  64. Edwards MSB, Wara WM, Urtasun RC, et al. Hyperfractionated radiotherapy for brain-stem glioma: A phase I-II trial. J Neurosurg 1989;70:691-700.
  65. Prados MD, Wara WM, Edwards MSB, et al. The treatment of brain stem and thalamic gliomas with 78 Gy of hyperfractionated radiation therapy. Int J RadiatOncolBiol Phys 1995;32:85-91.
  66. Stupp R, Mason WP, van den Bent MJ, et al. European Organisation for Research and Treatment of Cancer Brain Tumor and Radiotherapy Groups; National Cancer Institute of Canada Clinical Trials Group. Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma.NEngl J Med 2005;352(10):987-96.
  67. Zarghooni M, Bartels U, Lee E, et al. Whole-genome profiling of pediatric diffuse intrinsic pontine gliomas highlights platelet-derived growth factor receptor alpha and poly (ADP-ribose) polymerase as potential therapeutic targets. J ClinOncol 2010;28:1337-44.
  68. Gilbertson RJ, Hill DA, Hernan R, et al. ERBB1 is amplified and overexpressed in high-grade diffusely infiltrative pediatric brain stem glioma. Clin Cancer Res 2003;9:3620-4.
  69. Pollack IF, Stewart CF, Kocak M, et al. A phase II study of gefitinib and irradiation in children with newly diagnosed brainstem gliomas: a report from the Pediatric Brain Tumor Consortium. Neuro Oncol 2011;13:290-7.
  70. Haas-Kogan DA, Banerjee A, Poussaint TY, et al. Phase II trial of tipifarnib and radiation in children with newly diagnosed diffuse intrinsic pontine gliomas. Neuro Oncol 2011;13:298-306.
  71. Turner CD, Chi S, Marcus KJ, et al. Phase II study of thalidomide and radiation in children with newly diagnosed brainstem gliomas and glioblastoma multiforme. J Neurooncol 2007;82(1):95-101.
  72. Kim CY, Kim SK, Phi JH,, et al. A prospective study of temozolomide plus thalidomde during and after radiation therapy for pediatric diffuse pontine gliomas: preliminary results of the Korean Society for Pediatric Neuro-Oncology study. J Neurooncol 2010;100(2):193-8.
  73. Kivivuori SM, Riikonen P, Valanne L, et al. Antiangiogenic combination therapy after local radiotherapy with topotecanradiosensitizer improved quality of life for children with inoperable brainstem gliomas. ActaPediatr 2011;100(1):134-8.
  74. Broniscer A, Baker JN, Tagen M, et al. Phase I study of vandetanib during and after radiotherapy in children with diffuse intrinsic pontine glioma. J ClinOncol 2010;28(31):4762-8.
  75. Pollack IF, Jakacki RI, Blaney SM, et al. Phase I trial of imatinib in children with newly diagnosed brainstem and recurrent malignant gliomas: a Pediatric Brain Tumor Consortium report. Neuro Oncol 2007;9(2):145-60.
  76. Mundinger F, Braus DF, Krauss JK, Birg W. Long-term outcome of 89 low-grade brain-stem gliomas after interstitial radiation therapy. J Neurosurg 1991;75:740-6.
  77. Chuba PJ, Zamarano L, Hamre M, et al. Permanent I-125 brain stem implants in children. Childs NervSyst 1998;14:570-7.
  78. Yen CP, Sheehan J, Steiner M, et al. Gamma knife surgery for focal brainstem gliomas. J Neurosurg 2007;106:8-17
  79. Fuchs I, Kreil W, Sutter B, et al. Gamma Knife radiosurgery of brainstem gliomas.ActaNeurochirSuppl 2002;84:85-90.
  80. Milstein JM, Geyer JR, Berger MS et al. Favorable prognosis for brain stem gliomas in neurofibromatosis. J Neurooncol 1989;7:367-71.
  81. Pollack IF, Shultz B, Mulvihill JJ. The management of brain stem gliomas in patients with neurofibromatosis. Neurology 1996;46:1652-60.
  82. Fine VH, Barker GF II, Market MJ, et al. Neoplasms of the central nervous system. In de Vita VT Jr, Hellman S, Steven A, editors: Cancer Principles and Practice of Oncology, 7th ed, 2005, JB Lippincott, pp1834-1932.
  83. Shrieve DC, Wara WM, Edwards MS, et al. Hyperfractionated radiation therapy for gliomas of the brainstem in children and in adults.Int J RadiatOncolBiol Phys 1992;24:599-610.
  84. Guillamo JS, Monjour A, Taillandier L, et al. Brainstem gliomas in adults: prognostic factors and classification. Brain 2001;124:2528-39.
  85. Wolff JE, Rytting ME, Vats TS, et al. Treatment of recurrent diffuse intrinsic pontine glioma: the MD Anderson Cancer Center experience. J Neorooncol 2012;106(2):391-7.
  86. Warren KE, Gururangan S, Geyer JR, et al. A phase II study of O6-benzylguanine and temozolomide in pediatric patients with recurrent or progressive high-grade gliomas and brainstem gliomas: a Pediatric Brain Tumor Consortium study. J Neurooncol 2012; 106(3):643-9.
  87. Gururangan S, Chi SN, Young Poussaint T, et al. Lack of efficacy of bevacizumab plus irinotecan in children with recurrent malignant glioma and diffuse brainste glioma: a Pediatric Brain Tumor Consortium study. J ClinOncol 2010;28(18):3069-75
  88. Minturn JE, Janss AJ, Fisher PG, et al. A phase II study of metronomic oral topotecan for recurrent chilhood brain tumors. Pediatr Blood Cancer 2011;56(1):39-44.
  89. Hargrave D, Bartels U, Bouffet E. Diffuse brainstem glioma in children: critical review of clinical trials. Lancet Oncol 2006;7:241-8.
  90. Jansen MH, van Vuurden DG, Vandertop WP, et al. Diffuse intrinsic pontine gliomas: A systematic update on clinical trials and biology. Cancer Treat Rev 2012;38(1):27-35.

[Berrin Pehlivan, Ahmet Kucuk, Huseyin Mertsoylu and Erkan Topkan. (2019); CURRENT MANAGEMENT OF BRAINSTEM GLIOMAS. Int. J. of Adv. Res. 7 (Sep). 311-320] (ISSN 2320-5407). www.journalijar.com

Erkan Topkan
Baskent University, Department of Radiation Oncology

DOI:

Article DOI: 10.21474/IJAR01/9664      
DOI URL: https://dx.doi.org/10.21474/IJAR01/9664

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