INFRATENTORIAL BRAIN TUMORS

David I. Sandberg, M.D.

Epidemiology

Table 1: Relative Incidence of the Most Common Infratentorial Brain Tumors by Histology (Excluding Intrinsic Brainstem Tumors) in Children (34)

Tumor Histology | Percentage of Tumors
Medulloblastoma (PNET) | 30-55%
Cerebellar Astrocytoma | 25%
Ependymoma | 20%

Table 2: Uncommon Posterior Fossa Tumors in Children (34)

Hemangioblastoma
Choroid Plexus Pappilloma
Gaglioglioma
Dermoid
Epidermoid
Atypical Teratoid/Rhabdoid
Sarcoma
Acoustic Neuroma
Fibrosarcoma
Meningioma
Chordoma
Subependymoma
Metastasis (most common: neuroblastoma, embryonal rhabdomyosarcoma, Wilms tumor)

Presentation

General Management Principles

Preoperative Imaging Studies:

Management of Hydrocephalus Prior to Tumor Resection:

Management of Hydrocephalus After Tumor Resection:

Other Preoperative Considerations:

Postoperative Management:

Surgical Approaches

General Intraoperative Considerations

  1. Extreme caution must be taken when opening the dura in the posterior fossa, particularly in young children who may have enlarged dural venous sinuses.
  2. Tumor bleeding during resection can also be extensive, particularly for malignant tumors such as medulloblastoma.
  3. All patients should have an arterial line and appropriate venous access. In the event of extensive blood loss, coagulopathy and thrombocytopenia should be ruled out and aggressively corrected if diagnosed.
Midline Suboccipital Craniotomy: the common surgical procedure performed for children with infratentorial tumors. The basic surgical steps for a midline suboccipital craniotomy for tumor resection are outlined below: Other Surgical Approaches: Cerebello-pontine angle tumors in children, such as ependymomas, may be approached via a retrosigmoid approach as reviewed by Sanford et al. (61) . Focal brainstem tumors are best approached by the route which provides the shortest trajectory to the tumor. Surgical approaches to various areas within the brainstem are detailed in other references (52).

Complications of Surgery

Classification and Management of Specific Tumor Subtypes

Medulloblastoma

Table 3: High Risk Versus Average Risk Medulloblastoma (42)

Average Risk High Risk

Ependymoma

Cerebellar Astrocytoma

Brainstem Gliomas

Diffuse Tumors Dorsally Exophytic Tumors Cervicomedullary Tumors Focal Intrinsic Tumors Tectal Tumors

References

  1. Aggarwal R, Yeung D, Kumar P, Muhlbauer M, Kun LE: Efficacy and feasibility of stereotactic radiosurgery in the primary management of unfavorable pediatric ependymoma. Radiother Oncol 43:269-273, 1997.
  2. Albright AL, Guthkelch AN, Packer RJ, Price RA, Rourke LB: Prognostic factors in pediatric brain-stem gliomas. J Neurosurg 65:751-755, 1986.
  3. Albright AL, Packer RJ, Zimmerman R, Rorke LB, Boyett J, Hammond GD: Magnetic resonance scans should replace biopsies for the diagnosis of diffuse brain stem gliomas: a report from the Children's Cancer Group. Neurosurgery 33:1026-1029; discussion 1029-1030, 1993.
  4. Albright AL, Wisoff JH, Zeltzer PM, Boyett JM, Rorke LB, Stanley P: Effects of medulloblastoma resections on outcome in children: a report from the Children's Cancer Group. Neurosurgery 38:265-271, 1996.
  5. Bailey CC, Gnekow A, Wellek S, Jones M, Round C, Brown J, Phillips A, Neidhardt MK: Prospective randomised trial of chemotherapy given before radiotherapy in childhood medulloblastoma. International Society of Paediatric Oncology (SIOP) and the (German) Society of Paediatric Oncology (GPO): SIOP II. Med Pediatr Oncol 25:166-178, 1995.
  6. Berger MS, Edwards MS, LaMasters D, Davis RL, Wilson CB: Pediatric brain stem tumors: radiographic, pathological, and clinical correlations. Neurosurgery 12:298-302, 1983.
  7. Campbell JW, Pollack IF: Cerebellar astrocytomas in children. J Neurooncol 28:223-231, 1996.
  8. Chatty EM, Earle KM: Medulloblastoma. A report of 201 cases with emphasis on the relationship of histologic variants to survival. Cancer 28:977-983, 1971.
  9. Chiu JK, Woo SY, Ater J, Connelly J, Bruner JM, Maor MH, van Eys J, Oswald MJ, Shallenberger R: Intracranial ependymoma in children: analysis of prognostic factors. J Neurooncol 13:283-290, 1992.
  10. Desai KI, Nadkarni TD, Muzumdar DP, Goel A: Prognostic factors for cerebellar astrocytomas in children: a study of 102 cases. Pediatr Neurosurg 35:311-317, 2001.
  11. Due-Tonnessen BJ, Helseth E, Scheie D, Skullerud K, Aamodt G, Lundar T: Long-term outcome after resection of benign cerebellar astrocytomas in children and young adults (0-19 years): report of 110 consecutive cases. Pediatr Neurosurg 37:71-80, 2002.
  12. Duffner PK, Krischer JP, Sanford RA, Horowitz ME, Burger PC, Cohen ME, Friedman HS, Kun LE: Prognostic factors in infants and very young children with intracranial ependymomas. Pediatr Neurosurg 28:215-222, 1998.
  13. Dunkel IJ, Boyett JM, Yates A, Rosenblum M, Garvin JH, Jr., Bostrom BC, Goldman S, Sender LS, Gardner SL, Li H, Allen JC, Finlay JL: High-dose carboplatin, thiotepa, and etoposide with autologous stem-cell rescue for patients with recurrent medulloblastoma. Children's Cancer Group. J Clin Oncol 16:222-228, 1998.
  14. Epstein F, Wisoff J: Intra-axial tumors of the cervicomedullary junction. J Neurosurg 67:483-487, 1987.
  15. Epstein FJ, Farmer JP: Brain-stem glioma growth patterns. J Neurosurg 78:408-412, 1993.
  16. Farmer JP, McNeely PD, Freeman CR: Brainstem gliomas, in Albright AL, Pollack IF, Adelson PD (eds): Principles and Practice of Pediatric Neurosurgery. New York, Thieme Medical Publishers, Inc., 2008, pp 640-652.
  17. Farmer JP, Montes JL, Freeman CR, Meagher-Villemure K, Bond MC, O'Gorman AM: Brainstem Gliomas. A 10-year institutional review. Pediatr Neurosurg 34:206-214, 2001.
  18. Fernandez C, Figarella-Branger D, Girard N, Bouvier-Labit C, Gouvernet J, Paz Paredes A, Lena G: Pilocytic astrocytomas in children: prognostic factors--a retrospective study of 80 cases. Neurosurgery 53:544-553; discussion 554-545, 2003.
  19. Foreman NK, Love S, Thorne R: Intracranial ependymomas: analysis of prognostic factors in a population-based series. Pediatr Neurosurg 24:119-125, 1996.
  20. Fritsch MJ, Doerner L, Kienke S, Mehdorn HM: Hydrocephalus in children with posterior fossa tumors: role of endoscopic third ventriculostomy. J Neurosurg 103:40-42, 2005.
  21. Goldstein AM, Yuen J, Tucker MA: Second cancers after medulloblastoma: population-based results from the United States and Sweden. Cancer Causes Control 8:865-871, 1997.
  22. Hadjipanayis CG, Kondziolka D, Gardner P, Niranjan A, Dagam S, Flickinger JC, Lunsford LD: Stereotactic radiosurgery for pilocytic astrocytomas when multimodal therapy is necessary. J Neurosurg 97:56-64, 2002.
  23. Halberg FE, Wara WM, Fippin LF, Edwards MS, Levin VA, Davis RL, Prados MB, Wilson CB: Low-dose craniospinal radiation therapy forstem gliomas. J Neurosurg 65:751-755, 1986.2.Albright AL, Guthkelch AN, Packer RJ, Price RA, Rourke LB: Prognostic factors in pediatric brain-> medulloblastoma. Int J Radiat Oncol Biol Phys 20:651-654, 1991.
  24. Healey EA, Barnes PD, Kupsky WJ, Scott RM, Sallan SE, Black PM, Tarbell NJ: The prognostic significance of postoperative residual tumor in ependymoma. Neurosurgery 28:666-671; discussion 671-662, 1991.
  25. Hershatter BW, Halperin EC, Cox EB: Medulloblastoma: the Duke University Medical Center experience. Int J Radiat Oncol Biol Phys 12:1771-1777, 1986.
  26. Hoffman HJ: Dorsally exophytic brain stem tumors and midbrain tumors. Pediatr Neurosurg 24:256-262, 1996.
  27. Horn B, Heideman R, Geyer R, Pollack I, Packer R, Goldwein J, Tomita T, Schomberg P, Ater J, Luchtman-Jones L, Rivlin K, Lamborn K, Prados M, Bollen A, Berger M, Dahl G, McNeil E, Patterson K, Shaw D, Kubalik M, Russo C: A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol 21:203-211, 1999.
  28. Ilgren EB, Stiller CA: Cerebellar astrocytomas. Clinical characteristics and prognostic indices. J Neurooncol 4:293-308, 1987.
  29. Johnson DL, McCabe MA, Nicholson HS, Joseph AL, Getson PR, Byrne J, Brasseux C, Packer RJ, Reaman G: Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80:1004-1010, 1994.
  30. Kellogg JX, Piatt JH, Jr.: Resection of fourth ventricle tumors without splitting the vermis: the cerebellomedullary fissure approach. Pediatr Neurosurg 27:28-33, 1997.
  31. Kimmings E, Kleinlugtebeld AT, Casey A, Hayward RD: Medulloblastoma: factors influencing the educational potential of surviving children. Br J Neurosurg 9:611-617, 1995.
  32. Lee M, Wisoff JH, Abbott R, Freed D, Epstein FJ: Management of hydrocephalus in children with medulloblastoma: prognostic factors for shunting. Pediatr Neurosurg 20:240-247, 1994.
  33. Littman P, Jarrett P, Bilaniuk LT, Rorke LB, Zimmerman RA, Bruce DA, Carabell SC, Schut L: Pediatric brain stem gliomas. Cancer 45:2787-2792, 1980.
  34. Maher CO, Friedman JA, Raffel C: Posterior Fossa Tumors in Children, in Batjer HH, Loftus CM (eds): Textook of Neurological Surgery: Principles and Practice. Philadelphia, Lippincott Williams & Wilkins, 2003, p 985.
  35. Maksoud YA, Hahn YS, Engelhard HH: Intracranial ependymoma. Neurosurg Focus 13:e4, 2002.
  36. May PL, Blaser SI, Hoffman HJ, Humphreys RP, Harwood-Nash DC: Benign intrinsic tectal "tumors" in children. J Neurosurg 74:867-871, 1991.
  37. Mehta V, Chapman A, McNeely PD, Walling S, Howes WJ: Latency between symptom onset and diagnosis of pediatric brain tumors: an Eastern Canadian geographic study. Neurosurgery 51:365-372; discussion 372-363, 2002.
  38. Mesiwala AH, Avellino AM, Roberts TS, Ellenbogen RG: Spontaneous cerebellar hemorrhage due to a juvenile pilocytic astrocytoma: case report and review of the literature. Pediatr Neurosurg 34:235-238, 2001.
  39. Meyers SP, Kemp SS, Tarr RW: MR imaging features of medulloblastomas. AJR Am J Roentgenol 158:859-865, 1992.
  40. Muraszko KM, Brahma B, Orringer D: Medulloblastomas, in Albright AL, Pollack IF, Adelson PD (eds): Principles and Practice of Pediatric Neurosurgery. New York, Thieme Medical Publishers, Inc., 2008, pp 605, 606.
  41. Ogilvy-Stuart AL, Clayton PE, Shalet SM: Cranial irradiation and early puberty. J Clin Endocrinol Metab 78:1282-1286, 1994.
  42. Packer RJ, Cogen P, Vezina G, Rorke LB: Medulloblastoma: clinical and biologic aspects. Neuro Oncol 1:232-250, 1999.
  43. Packer RJ, Goldwein J, Nicholson HS, Vezina LG, Allen JC, Ris MD, Muraszko K, Rorke LB, Wara WM, Cohen BH, Boyett JM: Treatment of children with medulloblastomas with reduced-dose craniospinal radiation therapy and adjuvant chemotherapy: A Children's Cancer Group Study. J Clin Oncol 17:2127-2136, 1999.
  44. Pagni CA, Giordana MT, Canavero S: Benign recurrence of a pilocytic cerebellar astrocytoma 36 years after radical removal: case report. Neurosurgery 28:606-609, 1991.
  45. Perilongo G, Massimino M, Sotti G, Belfontali T, Masiero L, Rigobello L, Garre L, Carli M, Lombardi F, Solero C, Sainati L, Canale V, del Prever AB, Giangaspero F, Andreussi L, Mazza C, Madon E: Analyses of prognostic factors in a retrospective review of 92 children with ependymoma: Italian Pediatric Neuro-oncology Group. Med Pediatr Oncol 29:79-85, 1997.
  46. Pollack IF: Brain tumors in children. N Engl J Med 331:1500-1507, 1994.
  47. Pollack IF: Pediatric brain tumors. Semin Surg Oncol 16:73-90, 1999.
  48. Pollack IF: Intrinsic Tumors of the Brainstem, in Batjer HH, Loftus CM (eds): Textbook of Neurological Surgery: Principles and Practice. Philadelphia, Lippincott Williams & Wilkins, 2003, pp 974-984.
  49. Pollack IF, Hoffman HJ, Humphreys RP, Becker L: The long-term outcome after surgical treatment of dorsally exophytic brain-stem gliomas. J Neurosurg 78:859-863, 1993.
  50. Pollack IF, Pang D, Albright AL: The long-term outcome in children with late-onset aqueductal stenosis resulting from benign intrinsic tectal tumors. J Neurosurg 80:681-688, 1994.
  51. Pompili A, Caperle M, Pace A, Ramazzotti V, Raus L, Jandolo B, Occhipinti E: Quality-of-life assessment in patients who had been surgically treated for cerebellar pilocytic astrocytoma in childhood. J Neurosurg 96:229-234, 2002.
  52. Porter RW, Detwiler PW, Spetzler RF: Surgical Approaches to the Brainstem, in Spetzler RF (ed): Operative Techniques in Neurosurgery, W.B. Saunders Company, 2000, pp 114-123.
  53. Rezai AR, Woo HH, Lee M, Cohen H, Zagzag D, Epstein FJ: Disseminated ependymomas of the central nervous system. J Neurosurg 85:618-624, 1996.
  54. Roberts RO, Lynch CF, Jones MP, Hart MN: Medulloblastoma: a population-based study of 532 cases. J Neuropathol Exp Neurol 50:134-144, 1991.
  55. Robertson PL, Muraszko KM, Brunberg JA, Axtell RA, Dauser RC, Turrisi AT: Pediatric midbrain tumors: a benign subgroup of brainstem gliomas. Pediatr Neurosurg 22:65-73, 1995.
  56. Robertson PL, Zeltzer PM, Boyett JM, Rorke LB, Allen JC, Geyer JR, Stanley P, Li H, Albright AL, McGuire-Cullen P, Finlay JL, Stevens KR, Jr., Milstein JM, Packer RJ, Wisoff J: Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children's Cancer Group. J Neurosurg 88:695-703, 1998.
  57. Rutka JT, Kuo JS: Pediatric surgical neuro-oncology: current best care practices and strategies. J Neurooncol 69:139-150, 2004.
  58. Rutkowski S, Bode U, Deinlein F, Ottensmeier H, Warmuth-Metz M, Soerensen N, Graf N, Emser A, Pietsch T, Wolff JE, Kortmann RD, Kuehl J: Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med 352:978-986, 2005.
  59. Sainte-Rose C, Cinalli G, Roux FE, Maixner R, Chumas PD, Mansour M, Carpentier A, Bourgeois M, Zerah M, Pierre-Kahn A, Renier D: Management of hydrocephalus in pediatric patients with posterior fossa tumors: the role of endoscopic third ventriculostomy. J Neurosurg 95:791-797, 2001.
  60. Sanford RA, Gajjar A: Ependymomas. Clin Neurosurg 44:559-570, 1997.
  61. Sanford RA, Kun LE, Heideman RL, Gajjar A: Cerebellar pontine angle ependymoma in infants. Pediatr Neurosurg 27:84-91, 1997.
  62. Schoenberg BS, Schoenberg DG, Christine BW, Gomez MR: The epidemiology of primary intracranial neoplasms of childhood. A population study. Mayo Clin Proc 51:51-56, 1976.
  63. Smoots DW, Geyer JR, Lieberman DM, Berger MS: Predicting disease progression in childhood cerebellar astrocytoma. Childs Nerv Syst 14:636-648, 1998.
  64. Steinbok P, Cochrane DD, Perrin R, Price A: Mutism after posterior fossa tumour resection in children: incomplete recovery on long-term follow-up. Pediatr Neurosurg 39:179-183, 2003.
  65. Stroink AR, Hoffman HJ, Hendrick EB, Humphreys RP, Davidson G: Transependymal benign dorsally exophytic brain stem gliomas in childhood: diagnosis and treatment recommendations. Neurosurgery 20:439-444, 1987.
  66. Tamura M, Zama A, Kurihara H, Fujimaki H, Imai H, Kano T, Saitoh F: Management of recurrent pilocytic astrocytoma with leptomeningeal dissemination in childhood. Childs Nerv Syst 14:617-622, 1998.
  67. Taylor MD, Sanford RA, Boop FA: Cerebellar Pilocytic Astrocytomas, in Albright AL, Pollack IF, Adelson PD (eds): Principles and Practice of Pediatric Neurosurgery. New York, Thieme Medical Publishers, Inc., 2008, pp 655-666.
  68. van Veelen-Vincent ML, Pierre-Kahn A, Kalifa C, Sainte-Rose C, Zerah M, Thorne J, Renier D: Ependymoma in childhood: prognostic factors, extent of surgery, and adjuvant therapy. J Neurosurg 97:827-835, 2002.
  69. Vandertop WP, Hoffman HJ, Drake JM, Humphreys RP, Rutka JT, Amstrong DC, Becker LE: Focal midbrain tumors in children. Neurosurgery 31:186-194, 1992.
  70. Vinchon M, Ruchoux MM, Soto-Ares G, Nicolas R, Dhellemmes P: Cerebellar gliomas in infants: specificity, pathology, and outcome. Childs Nerv Syst 17:31-36, 2001.
  71. Woo C, Stea B, Lulu B, Hamilton A, Cassady JR: The use of stereotactic radiosurgical boost in the treatment of medulloblastomas. Int J Radiat Oncol Biol Phys 37:761-764, 1997.
  72. Yates AJ, Becker LE, Sachs LA: Brain tumors in childhood. Childs Brain 5:31-39, 1979.