Child's Nervous System

, Volume 20, Issue 3, pp 143–153 | Cite as

Brainstem gliomas

  • George I. Jallo
  • Ann Biser-Rohrbaugh
  • Diana Freed
Review Paper



Brainstem gliomas have historically been one of the most difficult pediatric cancers to treat. Tumors arising in the brainstem were once uniformly discounted as surgically unresectable lesions. Early neurosurgeons thought this location to be inoperable and fraught with disaster. The advent of computed tomography (CT), magnetic resonance imaging (MRI), and sophisticated neurophysiological monitoring techniques have significantly advanced the surgical treatment of these precarious lesions.


Brainstem gliomas are now recognized as a heterogenous group of tumors. They have been broadly classified into several categories depending upon the classification scheme. All these classification systems provide a framework to predict growth patterns, surgical resectability, and overall prognosis of these tumors. These systems allow the surgeon to obtain a better understanding of the distinction between low-grade tumors and diffuse inoperable tumor types. The authors review the current literature and management of brainstem tumors.


Brainstem glioma Cervicomedullary Midbrain tumors Pontine glioma Surgery 


  1. 1.
    Abbott R (1993) Tumors of the medulla. Neurosurg Clin N Am 4:519–527PubMedGoogle Scholar
  2. 2.
    Abbott R, Shiminski-Maher T, Wisoff JH, Epstein FJ (1991) Intrinsic tumors of the medulla: surgical complications. Pediatr Neurosurg 17:239–244PubMedGoogle Scholar
  3. 3.
    Abbott R, Feldstein N, Wisoff JH, Epstein FJ (1992) Osteoplastic laminotomy in children. Pediatr Neurosurg 18:153–156PubMedGoogle Scholar
  4. 4.
    Abbott R, Shiminski-Maher T, Epstein FJ (1996) Intrinsic tumors of the medulla: predicting outcome after surgery. Pediatr Neurosurg 25:41–44PubMedGoogle Scholar
  5. 5.
    Albright AL (1996) Brain stem gliomas. In: Youmans J (ed) Neurological surgery. Saunders, Philadelphia, pp 2603–2611Google Scholar
  6. 6.
    Albright AL, Guthkelch AN, Packer RJ, Price RA, Rourke LB (1986) Prognostic factors in pediatric brain-stem gliomas. J Neurosurg 65:751–755PubMedGoogle Scholar
  7. 7.
    Albright AL, Packer RJ, Zimmerman R, Rorke LB, Boyett J, Hammond GD (1993) Magnetic resonance scans should replace biopsies for the diagnosis of diffuse brain stem gliomas: a report from the Children’s Cancer Group. Neurosurgery 33:1026–1030PubMedGoogle Scholar
  8. 8.
    Allen J (1983) Brain stem glioma. Neurol Neurosurg Update Series 4:2–7Google Scholar
  9. 9.
    Allen JC, Siffert J (1996) Contemporary chemotherapy issues for children with brainstem gliomas. Pediatr Neurosurg 24:98–102PubMedGoogle Scholar
  10. 10.
    Alvisi C, Cerisoli M, Maccheroni ME (1985) Long-term results of surgically treated brainstem gliomas. Acta Neurochir 76:12–17Google Scholar
  11. 11.
    Ammirati M, Bernardo A, Musumeci A, Bricolo A (2002) Comparison of different infratentorial-supracerebellar approaches to the posterior and middle incisural space: a cadaveric study. J Neurosurg 97:922–928PubMedGoogle Scholar
  12. 12.
    Barkovich AJ, Krischer J, Kun LE, Packer R, Zimmermann RA, Freeman CR, Wara WM, Albright L, Allen JC, Hoffman HJ (1990) Brain stem gliomas: a classification system based on magnetic resonance imaging. Pediatr Neurosurg 16:73–83PubMedGoogle Scholar
  13. 13.
    Behnke J, Christen HJ, Bruck W, Markakis E (1997) Intra-axial endophytic tumors in the pons and/or medulla oblongata. I. Symptoms, neuroradiological findings, and histopathology in 30 children. Childs Nerv Syst 13:122–134CrossRefPubMedGoogle Scholar
  14. 14.
    Beltramello A, Lombardo MC, Masotto B, Bricolo A (2000) Imaging of brain stem tumors. Oper Tech Neurosurg 3:87–105Google Scholar
  15. 15.
    Berger MS, Edwards MS, LaMasters D, Davis RL, Wilson CB (1983) Pediatric brain stem tumors: radiographic, pathological, and clinical correlations. Neurosurgery 12:298–302PubMedGoogle Scholar
  16. 16.
    Blaney S, Berg SL, Pratt C, Weitman S, Sullivan J, Luchtman-Jones L, Bernstein M (2001) A phase I study of irinotecan in pediatric patients: a Pediatric Oncology Group study. Clin Cancer Res 7:32–37PubMedGoogle Scholar
  17. 17.
    Bowers DC, Georgiades C, Aronson LJ, Carson BS, Weingart JD, Wharam MD, Melhem ER, Burger PC, Cohen KJ (2000) Tectal gliomas: natural history of an indolent lesion in pediatric patients. Pediatr Neurosurg 32:24–29PubMedGoogle Scholar
  18. 18.
    Bowers DC, Krause TP, Aronson LJ, Barzi A, Burger PC, Carson BS, Weingart JD, Wharam MD, Melhem ER, Cohen KJ (2001) Second surgery for recurrent pilocytic astrocytoma in children. Pediatr Neurosurg 34:229–234CrossRefPubMedGoogle Scholar
  19. 19.
    Bricolo A (2000) Surgical management of intrinsic brain stem gliomas. Oper Tech Neurosurg 3:137–154Google Scholar
  20. 20.
    Broniscer A, Gajjar A, Bhargava R, Langston JW, Heideman R, Jones D, Kun LE, Taylor J (1997) Brain stem involvement in children with neurofibromatosis type 1: role of magnetic resonance imaging and spectroscopy in the distinction from diffuse pontine glioma. Neurosurgery 40:331–338PubMedGoogle Scholar
  21. 21.
    Broniscer A, Leite CC, Lanchote VL, Machado TM, Cristofani LM (2000) Radiation therapy and high-dose tamoxifen in the treatment of patients with diffuse brainstem gliomas: results of a Brazilian cooperative study. Brainstem Glioma Cooperative Group. J Clin Oncol 18:1246–1253PubMedGoogle Scholar
  22. 22.
    Carson BSS, Guarnieri M (2002) Local therapy for brain tumors. Adv Clin Neurosci 12:89–99Google Scholar
  23. 23.
    Choux M, Lena G, Do L (2000) Brainstem tumors. In: Choux M, Di Rocco C, Hockley A (eds) Pediatric neurosurgery. Churchill Livingstone, New York, pp 471–491Google Scholar
  24. 24.
    Constantini S, Epstein F (1996) Ultrasonic dissection. In: Rengachary SS (ed) Neurosurgery. McGraw-Hill, New York, pp 2714–2718Google Scholar
  25. 25.
    Dechambre S, Duprez T, Lecouvet F, Raftopoulos C, Gosnard G (1999) Diffusion-weighted MRI postoperative assessment of an epidermoid tumour in the cerebellopontine angle. Neuroradiology 41:829–831PubMedGoogle Scholar
  26. 26.
    Deletis V, Sala F, Morota N (2000) Intraoperative neurophysiological monitoring and mapping during brain stem surgery: a modern approach. Oper Tech Neurosurg 3:109–113Google Scholar
  27. 27.
    Epstein F (1985) A staging system for brainstem gliomas. Cancer 56:1804–1806PubMedGoogle Scholar
  28. 28.
    Epstein F, McCleary EL (1986) Intrinsic brain-stem tumors of childhood: surgical indications. J Neurosurg 64:11–15PubMedGoogle Scholar
  29. 29.
    Epstein F, Wisoff J (1987) Intra-axial tumors of the cervicomedullary junction. J Neurosurg 67:483–487PubMedGoogle Scholar
  30. 30.
    Epstein F, Wisoff JH (1988) Intrinsic brainstem tumors in childhood: surgical indications. J Neurooncol 6:309–317PubMedGoogle Scholar
  31. 31.
    Epstein FJ, Farmer JP (1993) Brain-stem glioma growth patterns. J Neurosurg 78:408–412PubMedGoogle Scholar
  32. 32.
    Epstein FJ, Ozek M (1993) The plated bayonet: a new instrument to facilitate surgery for intra-axial neoplasms of the spinal cord and brain stem. Technical note. J Neurosurg 78:505–507PubMedGoogle Scholar
  33. 33.
    Fischbein NJ, Prados MD, Wara W, Russo C, Edwards MS, Barkovich AJ (1996) Radiologic classification of brain stem tumors: correlation of magnetic resonance imaging appearance with clinical outcome. Pediatr Neurosurg 24:9–23PubMedGoogle Scholar
  34. 34.
    Freeman CR, Bourgouin PM, Sanford RA, Cohen ME, Friedman HS, Kun LE (1996) Long term survivors of childhood brain stem gliomas treated with hyperfractionated radiotherapy. Clinical characteristics and treatment related toxicities. The Pediatric Oncology Group. Cancer 77:555–562CrossRefPubMedGoogle Scholar
  35. 35.
    Freeman CR, Kepner J, Kun LE, Sanford RA, Kadota R, Mandell L, Friedman H (2000) A detrimental effect of a combined chemotherapy-radiotherapy approach in children with diffuse intrinsic brain stem gliomas? Int J Radiat Oncol Biol Phys 47:561–564CrossRefPubMedGoogle Scholar
  36. 36.
    Gilday DL, Ash J (1975) Accuracy of brain scanning in pediatric craniocerebral neoplasms. Radiology 117:93–97PubMedGoogle Scholar
  37. 37.
    Gnanalingham KK, Lafuente J, Thompson D, Harkness W, Hayward R (2002) Surgical procedures for posterior fossa tumors in children: does craniotomy lead to fewer complications than craniectomy? J Neurosurg 97:821–826PubMedGoogle Scholar
  38. 38.
    Hoffman HJ, Becker L, Craven MA (1980) A clinically and pathologically distinct group of benign brain stem gliomas. Neurosurgery 7:243–248PubMedGoogle Scholar
  39. 39.
    Jallo G, Kothbauer K, Epstein F (2000) Surgical management of cervicomedullary and dorsally exophytic brain stem tumors. Oper Tech Neurosurg 3:131–136Google Scholar
  40. 40.
    Jallo GI (2001) CUSA excel ultrasonic aspiration system. Neurosurgery 48:695–697PubMedGoogle Scholar
  41. 41.
    Jallo GI, Kothbauer KF, Epstein FJ (2002) Contact laser microsurgery. Childs Nerv Syst 18:333–336CrossRefPubMedGoogle Scholar
  42. 42.
    Kaplan AM, Albright AL, Zimmerman RA, Rorke LB, Li H, Boyett JM, Finlay JL, Wara WM, Packer RJ (1996) Brainstem gliomas in children. A Children’s Cancer Group review of 119 cases. Pediatr Neurosurg 24:185–192PubMedGoogle Scholar
  43. 43.
    Kedar A, Maria BL, Graham-Pole J, Ringdahl DM, Quisling RG, Mickle JP, Mendenhall NP, Marcus RB Jr, Gross S (1994) High-dose chemotherapy with marrow reinfusion and hyperfractionated irradiation for children with high-risk brain tumors. Med Pediatr Oncol 23:428–436PubMedGoogle Scholar
  44. 44.
    Khatib ZA, Heideman RL, Kovnar EH, Langston JA, Sanford RA, Douglas EC, Ochs J, Jenkins JJ, Fairclough DL, Greenwald C et al (1994) Predominance of pilocytic histology in dorsally exophytic brain stem tumors. Pediatr Neurosurg 20:2–10PubMedGoogle Scholar
  45. 45.
    Kurpad SN, Cohen AR (1999) Posterior fossa craniotomy: an alternative to craniectomy. Pediatr Neurosurg 31:54–57CrossRefPubMedGoogle Scholar
  46. 46.
    Littman P, Jarrett P, Bilaniuk LT, Rorke LB, Zimmerman RA, Bruce DA, Carabell SC, Schut L (1980) Pediatric brain stem gliomas. Cancer 45:2787–2792PubMedGoogle Scholar
  47. 47.
    Lonser RR, Walbridge S, Garmestani K, Butman JA, Walters HA, Vortmeyer AO, Morrison PF, Brechbiel MW, Oldfield EH (2002) Successful and safe perfusion of the primate brainstem: in vivo magnetic resonance imaging of macromolecular distribution during infusion. J Neurosurg 97:905–913PubMedGoogle Scholar
  48. 48.
    Matson D (1969) Tumors of the posterior fossa. In: Thomas C (ed) Neurosurgery of infancy and childhood. Thomas, Springfield, pp 469–477Google Scholar
  49. 49.
    Milstein JM, Geyer JR, Berger MS, Bleyer WA (1989) Favorable prognosis for brainstem gliomas in neurofibromatosis. J Neurooncol 7:367–371PubMedGoogle Scholar
  50. 50.
    Morota N, Deletis V, Epstein FJ, Kofler M, Abbott R, Lee M, Ruskin K (1995) Brain stem mapping: neurophysiological localization of motor nuclei on the floor of the fourth ventricle. Neurosurgery 37:922–930PubMedGoogle Scholar
  51. 51.
    Murakami N, Matsushima T, Kuba H, Ikezaki K, Morioka T, Mihara F, Inamura T, Fukui M (1999) Combining steady-state constructive interference and diffusion-weighted magnetic resonance imaging in the surgical treatment of epidermoid tumors. Neurosurg Rev 22:159–162CrossRefPubMedGoogle Scholar
  52. 52.
    Pollack IF, Hoffman HJ, Humphreys RP, Becker L (1993) The long-term outcome after surgical treatment of dorsally exophytic brain-stem gliomas. J Neurosurg 78:859–863PubMedGoogle Scholar
  53. 53.
    Pollack IF, Polinko P, Albright AL, Towbin R, Fitz C (1995) Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children: incidence and pathophysiology. Neurosurgery 37:885–893PubMedGoogle Scholar
  54. 54.
    Raffel C, McComb JG, Bodner S, Gilles FE (1989) Benign brain stem lesions in pediatric patients with neurofibromatosis: case reports. Neurosurgery 25:959–964PubMedGoogle Scholar
  55. 55.
    Rhoton AL (2000) Cerebellum and fourth ventricle. Neurosurgery (Supplement) 47:S7–S27Google Scholar
  56. 56.
    Robertson PL, Allen JC, Abbott IR, Miller DC, Fidel J, Epstein FJ (1994) Cervicomedullary tumors in children: a distinct subset of brainstem gliomas. Neurology 44:1798–1803PubMedGoogle Scholar
  57. 57.
    Rose E (1998) Aesthetic facial restoration. Lippincott-Raven, New YorkGoogle Scholar
  58. 58.
    Sandberg DI, Edgar MA, Souweidane MM (2002) Convection-enhanced delivery into the rat brainstem. J Neurosurg 96:885–891PubMedGoogle Scholar
  59. 59.
    Stein B (1971) The infratentorial supracerebellar approach to pineal lesions. J Neurosurg 35:197–202PubMedGoogle Scholar
  60. 60.
    Steinbok P, Cochrane DD, Perrin R, Price A (2003) Mutism after posterior fossa tumour resection in children: incomplete recovery on long-term follow-up. Pediatr Neurosurg 39:179–183CrossRefPubMedGoogle Scholar
  61. 61.
    Storm PB, Clatterbuck RE, Liu YJ, Johnson RM, Gillis EM, Guarnieri M, Carson BS (2003) A surgical technique for safely placing a drug delivery catheter into the pons of primates: preliminary results of carboplatin infusion. Neurosurgery 52:1169–1177PubMedGoogle Scholar
  62. 62.
    Stroink AR, Hoffman HJ, Hendrick EB, Humphreys RP, Davidson G (1987) Transependymal benign dorsally exophytic brain stem gliomas in childhood: diagnosis and treatment recommendations. Neurosurgery 20:439–444PubMedGoogle Scholar
  63. 63.
    Walker DA, Punt JA, Sokal M (1999) Clinical management of brain stem glioma. Arch Dis Child 80:558–564PubMedGoogle Scholar
  64. 64.
    Walter AW, Gajjar A, Ochs JS, Langston JW, Sanford RA, Kun LE, Heideman R (1998) Carboplatin and etoposide with hyperfractionated radiotherapy in children with newly diagnosed diffuse pontine gliomas: a phase I/II study. Med Pediatr Oncol 30:28–33PubMedGoogle Scholar
  65. 65.
    Wang G, Zhang J, Sun M, Wang C (2001) Surgical management of brainstem mass lesions: respiratory insufficiency occurrence and recovery. Neurosurg Q 11:302–313CrossRefGoogle Scholar
  66. 66.
    Weiner HL, Freed D, Woo HH, Rezai AR, Kim R, Epstein FJ (1997) Intra-axial tumors of the cervicomedullary junction: surgical results and long-term outcome. Pediatr Neurosurg 27:12–18PubMedGoogle Scholar
  67. 67.
    Wisoff JH, Epstein FJ (1984) Pseudobulbar palsy after posterior fossa operation in children. Neurosurgery 15:707–709PubMedGoogle Scholar
  68. 68.
    Zagzag D, Miller DC, Knopp E, Farmer JP, Lee M, Biria S, Pellicer A, Epstein FJ, Allen JC (2000) Primitive neuroectodermal tumors of the brainstem: Investigation of seven cases. Pediatrics 106:1045–1053PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • George I. Jallo
    • 1
  • Ann Biser-Rohrbaugh
    • 1
  • Diana Freed
    • 1
  1. 1.Division of Pediatric NeurosurgeryJohns Hopkins HospitalBaltimoreUSA

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