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Neuropsychological sequelae and quality of life following treatment of posterior fossa ependymomas in children

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Abstract

Introduction

Modern treatment of posterior fossa ependymomas in children has improved the overall survival, but as more children live longer, the neuropsychological problems that they endure as result of the disease and its treatment become more the focus of attention.

Material

Early problems such as cerebellar mutism appear to be related to surgery and do not seem to be influenced in their incidence or outcome by the otherwise beneficial effect of oncological treatment. Indeed, the cause of cerebellar mutism is still elusive. While it was originally believed to be totally reversible, it is increasingly realized that it may be related to more complex delayed neuropsychological problems. Late problems such as the cerebellar cognitive affective syndrome are increasingly being recognized. The deleterious effect of both surgery and radiotherapy that is commonly administered afterwards is now well appreciated.

Conclusion

Careful neuropsychological assessment of these children and early intervention will maximize chances for recovery and improvement of academic functions and quality of life issues.

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References

  1. Ater JL, Moore BD, Francis DJ, Castillo R, Slopis J, Copeland DR (1996) Correlation of medical and neurosurgical events with neuropsychological status in children at diagnosis of astrocytoma: utilization of a neurological severity score. J Child Neurol 11:462–469

    Article  PubMed  CAS  Google Scholar 

  2. Benesch M, Spiegl K, Winter A, Passini A, Lackner H, Moser A, Sovinz P, Schwinger W, Urban C (2009) A scoring system to quantify late effects in children after treatment for medulloblastoma/ependymoma and its correlation with quality of life and neurocognitive functioning. Childs Nerv Syst 25:173–181

    Article  PubMed  Google Scholar 

  3. Bhat SR, Goodwin TL, Burwinkle TM, Lansdale MF, Dahl GV, Huhn SL, Gibbs IC, Donaldson SS, Rosenblum RK, Varni JW, Fisher PG (2005) Profile of daily life in children with brain tumors: an assessment of health-related quality of life. J Clin Oncol 23:5492–5500

    Article  Google Scholar 

  4. Chapman CA, Waber DP, Gernstein JH, Pomeroy SL, LaVally B, Sallan SE, Tarbell N (1995) Neurobehavioral and neurologic outcome in long-term survivors of posterior fossa brain tumours: role of age and perioperative factors. J Child Neurol 10:209–212

    Article  PubMed  CAS  Google Scholar 

  5. Conklin HM, Li C, Xiong X, Ogg RJ, Merchant TE (2008) Predicting change in academic abilities after conformal radiation therapy for localized ependymoma. J Clin Oncol 26:3965–3970

    Article  PubMed  Google Scholar 

  6. Copeland DR, DeMoor C, Moore BD, Ater JL (1999) Neurocognitive development of children after a cerebellar tumor in infancy: a longitudinal study. J Clin Oncol 17(11):3476–3486

    PubMed  CAS  Google Scholar 

  7. Dennis M, Spiegler BJ, Hetherington CR, Greenberg ML (1996) Neuropsychological sequelae of the treatment of children with medulloblastoma. J Neurooncol 29:91–101

    Article  PubMed  CAS  Google Scholar 

  8. Dolan JR (1998) A cognitive affective role for the cerebellum (editorial). Brain 121:545–546

    Article  PubMed  Google Scholar 

  9. Ellenberg L, McComb JG, Siegel SE, Stowe S (1987) Factors affecting intellectual outcome in pediatric brain tumor patients. Neurosurgery 21:638–644

    Article  PubMed  CAS  Google Scholar 

  10. Frassanito P, Massimi L, Caldarelli M, Di Rocco C (2009) Cerebellar mutism after spontaneous intratumoral bleeding involving the upper cerebellar vermis: a contribution to the physiopathogenic interpretation. Childs Nerv Syst 25:7–11

    Article  PubMed  Google Scholar 

  11. George PA, Kuehn SM, Vassilyadi M, Richards PMP, Parlow SE, Keene DL, Ventureyra ECG (2003) Cognitive sequelae in children with posterior fossa tumors. Pediatr Neurol 28:42–47

    Article  PubMed  Google Scholar 

  12. Grill J, Renaux VK, Bulteau C, Viguier D, Levy-Piebois C, Saint-Rose C, Dellatollas G, Raquin MA, Jambaque I, Kalifa C (1999) Long-term intellectual outcome in children with posterior fossa tumors according to radiation doses and volumes. Int J Radiat Oncol Biol Phys 45:137–145

    PubMed  CAS  Google Scholar 

  13. Horn B, Heideman R, Geyer R, Pollack I, Packer R, Goldwein J, Tomita T, Schomberg P, Ater J, Lutchman-Jones L, Rivlin K, Lamborn K, Prados M, Bollen A, Berger M, Dahl G, McNeil E, Patterson K, Shaw D, Kubalic M, Russo C (1999) A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol 21(3):203–211

    Article  PubMed  CAS  Google Scholar 

  14. Huber JF, Bradley K, Spiegler BJ, Dennis M (2006) Long-term effects of transient cerebellar mutism after cerebellar astrocytoma or medulloblastoma tumor resection in childhood. Childs Nerv Syst 22:132–138

    Article  PubMed  Google Scholar 

  15. Huber JF, Bradley K, Spiegler B, Dennis M (2007) Long-term neuromotor speech deficits in survivors of childhood posterior fossa tumors: effects of tumor type, radiation, age at diagnosis and survival years. J Child Neurol 22(7):848–854

    Article  PubMed  Google Scholar 

  16. Karatekin C, Lazareff JA, Asarnow R (2000) Relevance of the cerebellar hemispheres for executive functions. Pediatr Neurol 22:106–112

    Article  PubMed  CAS  Google Scholar 

  17. Kingma A, Mooij JJA, Metzemaekers JDM, Leeuw JA (1994) Transient mutism and speech disorders after posterior fossa surgery in children with brain tumors. Acta Neurochir (Wien) 131:74–79

    Article  CAS  Google Scholar 

  18. LeBaron S, Zeltzer PM, Zeltzer LK et al (1988) Assessment of quality of survival in children with medulloblastoma and cerebellar astrocytoma. Cancer 62:1215–1222

    Article  PubMed  CAS  Google Scholar 

  19. Levisohn L, Cronin-Golomb A, Schmahmann JD (2000) Neuropsychological consequences of cerebellar tumour resection in children. Brain 123:1041–1050

    Article  PubMed  Google Scholar 

  20. Mabbott DJ, Spiegler BJ, Greenberg ML, Rutka JT, Hyder DJ, Bouffet E (2005) Serial evaluation of academic and behavioral outcome after treatment with cranial irradiation in childhood. J Clin Oncol 23:2256–2263

    Article  PubMed  Google Scholar 

  21. Maunsell E, Pogany L, Barrera M, Shaw AK, Speechley KN (2006) Quality of life among long-term adolescent and adult survivors of childhood cancer. J Clin Oncol 24:2527–2535

    Article  PubMed  Google Scholar 

  22. Merchant TE, Jenkins JJ, Burger PC, Sanford RA, Sherwood SH, Jones-Wallace D, Heideman RL, Thompson SJ, Helton KJ, Kun LE (2002) Influence of tumor grade on time to progression after irradiation for localized ependymoma in children. Int J Radiat Oncol Biol Phys 53:52–57

    Article  PubMed  Google Scholar 

  23. Merchant TE, Mulhern RK, Krasin MJ, Kun LE, Williams T, Li C, Xiong X, Khan RB, Lustig RH, Boop FA, Sanford RA (2004) Preliminary results from a phase II trial of conformal radiation therapy and evaluations of radiation-related CNS effects for pediatric patients with localised ependymoma. J Clin Oncol 22:3156–3162

    Article  PubMed  Google Scholar 

  24. Merchant TE, Kiehna EN, Li C, Xiong X, Mulhern RK (2005) Radiation dosimetry predicts IQ after conformal radiation therapy in pediatric patients with localised ependymoma. Int J Radiat Oncol Biol Phys 63:1546–1554

    Article  PubMed  Google Scholar 

  25. Mulhern RK, Williams JM, Lesure SS (1986) Neuropsychological performance of children surviving cerebellar tumors: six case studies. Int J Clin Neuropsychol 8:72–76

    Google Scholar 

  26. Mulhern RK, Kepner JL, Thomas PR, Armstrong FD, Friedman HS, Kun LE (1998) Neuropsychologic functioning of survivors of childhood medulloblastoma randomized to receive conventional or reduced dose craniospinal irradiation: a Pediatric Oncology Group study. J Clin Oncol 16:1723–1728

    PubMed  CAS  Google Scholar 

  27. Mulhern RK, Merchant TE, Gajjar A, Reddick WE, Kun LE (2004) Late neurocognitive sequelae in survivors of brain tumours in childhood. Lancet Oncol 5:399–408

    Article  PubMed  Google Scholar 

  28. Packer RJ (2008) Childhood brain tumors: accomplishments and ongoing challenges. J Child Neurol 23(10):1122–1127

    Article  PubMed  Google Scholar 

  29. Packer RJ, Sutton LN, Atkins TE, Radcliffe J, Bunin GR, D’Angio G, Siegel KR, Schut L (1989) A prospective study of cognitive function in children receiving whole-brain radiotherapy and chemotherapy: 2-year results. J Neurosurg 70:707–713

    Article  PubMed  CAS  Google Scholar 

  30. Peris-Bonet R, Martinez-Garcia C, Lacour B, Petrovich S, Giner-Ripoll B, Navajas A, Stelirova-Foucher E (2006) Childhood central nervous system tumours—incidence and survival in Europe (1978–1997): report from the Automated Childhood Cancer Information System project. Eur J Cancer 42:2064–2080

    Article  PubMed  Google Scholar 

  31. Ris MD, Packer R, Goldwein J, Jones-Wallace D, Boyett JM (2001) Intellectual outcome after reduced-dose radiation therapy plus adjuvant chemotherapy for medulloblastoma: a Children’s Cancer Group study. J Clin Oncol 19:3470–3476

    PubMed  CAS  Google Scholar 

  32. Riva D, Giorgi C (2000) The cerebellum contributes to higher functions during development: evidence from a series of children surgically treated for posterior fossa tumors. Brain 123:1051–1061

    Article  PubMed  Google Scholar 

  33. 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, Milstein JM, Packer RJ, Wisoff J, The Children’s Cancer Group (1998) 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

    Article  PubMed  CAS  Google Scholar 

  34. Rousseau P, Habrand JL, Sarrazin D, Kalifa C, Terrier-Lacombe MJ, Rekacewicz C, Rey A (1994) Treatment of intracranial ependymomas in children: review of a 15-year experience. Int J Radiat Oncol Biol Phys 28:381–386

    PubMed  CAS  Google Scholar 

  35. Schmahmann JD (1991) An emerging concept. The cerebellar contribution to higher function. Arch Neurol 48:1178–1187

    PubMed  CAS  Google Scholar 

  36. Schmahmann JD, Sherman JC (1998) The cerebellar cognitive affective syndrome. Brain 121:561–579

    Article  PubMed  Google Scholar 

  37. Seaver E, Geyer R, Sulzbacher S, Warner M, Batzel L, Milstein J, Berger M (1994) Psychosocial adjustment in long-term survivors of childhood medulloblastoma and ependymoma treated with craniospinal irradiation. Pediatr Neurosurg 20:248–253

    Article  PubMed  CAS  Google Scholar 

  38. Spiegler BJ, Bouffet E, Greenberg ML, Rutka JT, Mabbott DJ (2004) Change in neurocognitive functioning after treatment with cranial radiation in childhood. J Clin Oncol 22:706–713

    Article  PubMed  Google Scholar 

  39. Steinlin M, Imfeld S, Zulauf P, Boltshauser E, Lovblad KO, Luthy AR, Perrig W, Kaufmann F (2003) Neuropsychological long-term sequelae after posterior fossa tumour resection during childhood. Brain 126:1998–2008

    Article  PubMed  Google Scholar 

  40. Wells EM, Walsh KS, Khademian ZP, Keating RF, Packer RJ (2008) The cerebellar mutism syndrome and its relation to cerebellar cognitive function and the cerebellar cognitive affective disorder. Dev Disabil Res Rev 14:221–228

    Article  PubMed  Google Scholar 

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Correspondence to Spyros Sgouros.

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Charalambides, C., Dinopoulos, A. & Sgouros, S. Neuropsychological sequelae and quality of life following treatment of posterior fossa ependymomas in children. Childs Nerv Syst 25, 1313–1320 (2009). https://doi.org/10.1007/s00381-009-0927-2

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  • DOI: https://doi.org/10.1007/s00381-009-0927-2

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