Skip to main content
SpringerLink
Log in

A scoring system to quantify late effects in children after treatment for medulloblastoma/ependymoma and its correlation with quality of life and neurocognitive functioning

  • Original Paper
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Background

The aim of this study was to quantify the severity of late effects by a simple numerical score (late effects severity score, LESS) in patients who received radiochemotherapy for medulloblastoma or ependymoma. The LESS was correlated with neurocognitive and quality of life (QoL) outcomes.

Patients and methods

The LESS was calculated by assigning 0, 1, or 2 points within each of four different categories (neurology, endocrine, visual/auditory, others). Twenty-three patients with medulloblastoma (n = 18) or ependymoma (n = 5) underwent extensive neurocognitive and QoL testing at a median of 56 months (range, 1–174) after the end of treatment. Eight patients with low-grade glioma (LGG) who underwent tumor resection only served as control group.

Results

Patients with medulloblastoma/ependymoma had significantly higher LESS and significantly lower Wechsler Adult Intelligence Scale (WAIS)/Wechsler Intelligence Scales for Children (WISC) scores compared to patients with LGG. There was no statistically significant correlation between neurocognitive performance and QoL. The total LESS was negatively correlated with WAIS/WISC, attention, concentration, and verbal learning scores. Comparison of QoL and late effects in patients with medulloblastoma/ependymoma demonstrated a significant negative correlation only for neurological late effects and the KINDL score suggesting that younger patients with more severe late effects reported on a worse QoL.

Conclusions

This LESS seems to be a simple and practical tool to quantify late effects in former brain tumor patients. Although both groups differ significantly with regard to neurocognitive parameters and severity of late effects, this does not apply for all QoL outcomes. Neurological late effects seem to be most predictive for an impaired QoL in younger children.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Aaronson NK, Ahmedzai S, Bergman B, Bullinger M, Cull A, Duez NJ, Filiberti A, Flechtner H, Fleishman SB, de Haes JC, Kaasa S, Klee MC, Osoba D, Razavi D, Rofe PB, Schraub S, Sneeuw KC, Sullivan M, Takeda F (1993) The European Organization for Research and Treatment of Cancer QLQ-C30: a quality-of-life instrument for use in international clinical trials in oncology. J Natl Cancer Inst 85:365–376

    Article  PubMed  CAS  Google Scholar 

  2. Allen JC (1978) The effects of cancer therapy on the nervous system. J Pediatr 93:903–909

    Article  PubMed  CAS  Google Scholar 

  3. Benesch M, Lackner H, Sovinz P, Suppan E, Schwinger W, Eder HG, Dornbusch HJ, Moser A, Triebl-Roth K, Urban C (2006) Late sequela after treatment of childhood low-grade gliomas: a retrospective analysis of 69 long-term survivors treated between 1983 and 2003. J Neurooncol 78:199–205

    Article  PubMed  Google Scholar 

  4. 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 23:5493–5500

    Article  PubMed  Google Scholar 

  5. Brickenkamp R (1983) Test d2. Hogrefe, Göttingen

    Google Scholar 

  6. Buchanan GR, Adix L (2002) Grading of hemorrhage in children with idiopathic thrombocytopenic purpura. J Pediatr 141:683–688

    Article  PubMed  Google Scholar 

  7. Calaminus G, Kiebert G (1999) Studies on health-related quality of life in childhood cancer in the European setting: an overview. Int J Cancer (Suppl) 12:83–86

    Article  CAS  Google Scholar 

  8. Copeland DR, deMoor C, Moore BD 3rd, Ater JL (1999) Neurocognitive development of children after a cerebellar tumor in infancy: a longitudinal study. J Clin Oncol 17:3476–3486

    PubMed  CAS  Google Scholar 

  9. Danoff BF, Cowchock FS, Marquette C, Mulgrew L, Kramer S (1982) Assessment of the long-term effects of primary radiation therapy for brain tumors in children. Cancer 49:1580–1586

    Article  PubMed  CAS  Google Scholar 

  10. Gajjar A, Chintagumpala M, Ashley D, Kellie S, Kun LE, Merchant TE, Woo S, Wheeler G, Ahern V, Krasin MJ, Fouladi M, Broniscer A, Krance R, Hale GA, Stewart CF, Dauser R, Sanford RA, Fuller C, Lau C, Boyett JM, Wallace D, Gilbertson RJ (2006) Risk-adapted craniospinal radiotherapy followed by high-dose chemotherapy and stem-cell rescue in children with newly diagnosed medulloblastoma (St Jude Medulloblastoma-96): long-term results from a prospective, multicenter trial. Lancet Oncol 7:813–820

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  12. Greenberg P, Cox C, LeBeau MM, Fenaux P, Morel P, Sanz G, Sanz M, Vallespi T, Hamblin T, Oscier D, Ohyashiki K, Toyama K, Aul C, Mufti G, Bennett J (1997) International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 89:2079–2088

    PubMed  CAS  Google Scholar 

  13. Grill J, Renaux VK, Bulteau C, Viguier D, Levy-Piebois C, Sainte-Rose C, Dellatolas G, Raquin MA, Jambaqué 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 

  14. Helmstaedter C, Durwen HF (1990) VLMT: Verbaler Lern- und Merkfähigkeitstest. Ein praktikables und differenziertes Instrumentarium zur Prüfung der verbalen Gedächtnisleistung. Schweiz Arch Neurol Psychiatr 141:21–30

    PubMed  CAS  Google Scholar 

  15. Hoppe-Hirsch E, Renier D, Lellouch-Tubiana A, Sainte-Rose C, Pierre-Kahn A, Hirsch JF (1990) Medulloblastoma in childhood: progressive intellectual deterioration. Childs Nerv Syst 6:60–65

    Article  PubMed  CAS  Google Scholar 

  16. Horzic M, Kopljar M, Cupurdija K, Bielen DV, Vergles D, Lackovic Z (2007) Comparison of P-POSSUM and Cr-POSSUM scores in patients undergoing colorectal cancer resection. Arch Surg 142:1043–1048

    Article  PubMed  Google Scholar 

  17. Johnson DL, McCabe MA, Nicholson HS, Joseph AL, Getson PR, Byrne J, Brasseux C, Packer RJ, Reaman G (1994) Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80:1004–1010

    PubMed  CAS  Google Scholar 

  18. Kessler J, Schaaf A, Mielke R (1993) Der fragmentierte Bildertest. Hogrefe, Göttingen

    Google Scholar 

  19. Kleihues P, Burger PC, Scheithauer BW (1993) Histological typing of tumours of the central nervous system, 2nd edn. Springer, Berlin

    Google Scholar 

  20. Kortmann RD, Kühl J, Timmermann B, Mittler U, Urban C, Budach V, Richter E, Willich N, Flentje M, Berthold F, Slavc I, Wolff J, Meisner C, Wiestler O, Sörensen N, Warmuth-Metz M, Bamberg M (2000) Postoperative neoadjuvant chemotherapy before radiotherapy as compared to immediate radiotherapy followed by maintenance chemotherapy in the treatment of medulloblastoma in childhood: results of the German prospective randomized trial HIT ’91. Int J Radiat Oncol Biol Phys 46:269–279

    Article  PubMed  CAS  Google Scholar 

  21. Lackner H, Benesch M, Schagerl S, Kerbl R, Schwinger W, Urban C (2000) Prospective evaluation of late effects after childhood cancer therapy with a follow-up over 9 years. Eur J Pediatr 159:750–758

    Article  PubMed  CAS  Google Scholar 

  22. Li FP, Winston KR, Gimbrere K (1984) Follow-up of children with brain tumors. Cancer 54:135–138

    Article  PubMed  CAS  Google Scholar 

  23. Maddrey AM, Bergeron JA, Lombardo ER, McDonald NK, Mulne AF, Barenberg PD, Bowers DC (2005) Neuropsychological performance and quality of life of 10 year survivors of childhood medulloblastoma. J Neurooncol 72:245–253

    Article  PubMed  Google Scholar 

  24. 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 43:2527–2535

    Article  Google Scholar 

  25. Mostow EN, Byrne J, Connelly RR, Mulvihill JJ (1991) Quality of life in long-term survivors of CNS tumors of childhood and adolescence. J Clin Oncol 9:592–599

    PubMed  CAS  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 tumors in childhood. Lancet Oncol 5:399–408

    Article  PubMed  Google Scholar 

  28. Needle MN, Goldwein JW, Grass J, Cnaan A, Bergman I, Molloy P, Sutton L, Zhao H, Garvin JH Jr, Phillips PC (1997) Adjuvant chemotherapy for the treatment of intracranial ependymoma of childhood. Cancer 80:341–347

    Article  PubMed  CAS  Google Scholar 

  29. Packer RJ, Gurney JG, Punyko JA, Donaldson SS, Inskip PD, Stovall M, Yasui Y, Mertens AC, Sklar CA, Nicholson HS, Zeltzer LK, Neglia JP, Robison LL (2003) Long-term neurologic and neurosensory sequelae in adult survivors of a childhood brain tumor: Childhood Cancer Survivor Study. J Clin Oncol 21:3255–3261

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  31. Ravens-Sieberer U, Bullinger M (1998) Assessing health-related quality of life in chronically ill children with the German KINDL: first psychometric and content analytical results. Qual Life Res 7:399–407

    Article  PubMed  CAS  Google Scholar 

  32. Ribi K, Relly C, Landolt MA, Alber FD, Boltshauser MA, Grotzer MA (2005) Outcome of medulloblastoma in children: long-term complications and quality of life. Neuropediatrics 36:357–365

    Article  PubMed  CAS  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 Jr, Milstein JM, Packer RJ, Wisoff J (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 of children: review of a 15-year experience. Int J Radiat Oncol Biol Phys 28:381–386

    PubMed  CAS  Google Scholar 

  35. Tewes U (1994) Hamburg-Wechsler Intelligenztest für Erwachsene-Revision 1991. HAWIE-R. 2. korrigierte Auflage. Hans Huber, Bern

    Google Scholar 

  36. Tewes U (1983) Hamburg-Wechsler Intelligenztest für Kinder, Revision 1983. Hans Huber, Bern

    Google Scholar 

  37. Timmermann B, Kortmann RD, Kühl J, Meisner C, Slavc I, Pietsch T, Bamberg M (2000) Combined postoperative irradiation and chemotherapy for anaplastic ependymomas in childhood: results of the German prospective trials HIT 88/89 and HIT 91. Int J Radiat Oncol Biol Phys 46:287–295

    Article  PubMed  CAS  Google Scholar 

  38. Walter AW, Mulhern RK, Gajjar A, Heideman RL, Reardon D, Sanford RA, Xiong X, Kun LE (1999) Survival and neurodevelopmental outcome of young children with medulloblastoma at St Jude Children’s Research Hospital. J Clin Oncol 17:3720–3728

    PubMed  CAS  Google Scholar 

  39. Zeltzer PM, Boyett JM, Finlay JL, Albright AL, Rorke LB, Milstein JM, Allen JC, Stevens KR, Stanley P, Li H, Wisoff JH, Geyer JR, McGuire-Cullen P, Stehbens JA, Shurin SB, Packer RJ (1999) Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children’s Cancer Group 921 randomized phase III study. J Clin Oncol 17:832–838

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Auenbruggerplatz 30, 8036, Graz, Austria

    Martin Benesch, Katharina Spiegl, Anita Winter, Andrea Passini, Herwig Lackner, Andrea Moser, Petra Sovinz, Wolfgang Schwinger & Christian Urban

Authors
  1. Martin Benesch
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Katharina Spiegl
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Anita Winter
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Andrea Passini
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Herwig Lackner
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Andrea Moser
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Petra Sovinz
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Wolfgang Schwinger
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Christian Urban
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Martin Benesch.

Additional information

Katharina Spiegl and Anita Winter contributed equally to this work.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Benesch, M., Spiegl, K., Winter, A. et al. 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 (2009). https://doi.org/10.1007/s00381-008-0742-1

Download citation

  • Received:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-008-0742-1

Keywords

Search

Navigation