Subscribe to RSS
DOI: 10.1055/s-2005-872880
Georg Thieme Verlag KG Stuttgart · New York
Outcome of Medulloblastoma in Children: Long-Term Complications and Quality of Life
Publication History
Received: June 26, 2005
Accepted after Revision: September 7, 2005
Publication Date:
24 November 2005 (online)
Abstract
To study the outcomes in long-term survivors of paediatric medulloblastoma (MB), we followed 51 consecutive children who were treated between 1980 and 2000 in a single institution. In 18 of 26 survivors (mean follow-up time 12.2 years), tumour control, neurological, endocrine, and neurocognitive complications and their impact on behavioural and psychological adjustment, and health-related quality of life (QoL) were comprehensively assessed using qualitative and quantitative measures. Endocrine deficits occurred in 61 %, neurological complications in 72 %, and significant school problems in 72 %. All patients had significant deficits in neurocognitive functioning: attention and processing speed was impaired in 79 %, learning and memory in 88 %, language in 56 %, visual perception in 50 %, and executive functions in 64 %. In comparison with healthy controls, social functioning was rated by the patients as the QoL dimension most affected. Parents' ratings were considerably lower than those of the patients. No MB survivor > 18 years of age (n = 12) had a boy- or girlfriend. Because of their treatment, including craniospinal radiotherapy, MB long-time survivors are not only at great risk for neurological, endocrine, and neurocognitive complications, but also of social isolation thereby decreasing self-rated QoL substantially.
Key words
Brain tumour - medulloblastoma - primitive neuroectodermal brain tumour - psychological adjustment - quality of life - long-term complication
References
- 1 Achenbach T M. Manual for the Child Behavior Checklist 4 - 18 and 1991 profile. Burlington, VT; University of Vermont, Department of Psychiatry 1991
- 2 Achenbach T M. Manual for the Youth Self-Report and 1991 profile. Burlington, VT; University of Vermont, Department of Psychiatry 1991
- 3 Ater J L, van Eys J, Woo S Y, Moore 3rd B, Copeland D R, Bruner J. MOPP chemotherapy without irradiation as primary postsurgical therapy for brain tumors in infants and young children. J Neuro-Oncol. 1997; 32 243-252
- 4 Bradlyn A S, Ritchey A K, Harris C V, Moore I M, O'Brien R T. et al . Quality of life research in pediatric oncology. Research methods and barriers. Cancer. 1996; 78 1333-1339
- 5 Calaminus G, Weinspach S, Teske C, Gobel U. Quality of life in children and adolescents with cancer. First results of an evaluation of 49 patients with the PEDQOL questionnaire. Klin Padiatr. 2000; 212 211-215
- 6 Chapman C A, Waber D P, Bernstein J H, Pomeroy S L, LaVally B. et al . Neurobehavioral and neurologic outcome in long term survivors of posterior fossa brain tumors: role of age and perioperative factors. J Child Neurol. 1995; 10 209-212
- 7 Chin H W, Maruyama Y. Age at treatment and long-term performance results in medulloblastoma. Cancer. 1984; 53 1952-1958
- 8 Dennis M, Spiegler B J, Hetherington C R, Greenberg M L. Neuropsychological sequelae of the treatment of children with medulloblastoma. J Neuro-Oncol. 1996; 29 91-101
- 9 Duffner P K, Cohen M E, Thomas P. Late effects of treatment on the intelligence of children with posterior fossa tumors. Cancer. 1983; 51 233-237
- 10 Duffner P K, Horowitz M E, Krischer J P, Friedman H S, Burger P C. et al . Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. New Engl J Med. 1993; 328 1725-1731
- 11 Eiser C, Morse R. The measurement of quality of life in children: past and future perspectives. J Dev Behav Pediatr. 2001; 2 248-256
- 12 Felder-Puig R, Frey E, Proksch K, Varni J W, Gadner H, Topf R. Validation of the German version of the Pediatric Quality of Life Inventory (PedsQL) in childhood cancer patients off treatment and children with epilepsy. Qual Life Res. 2004; 13 223-234
- 13 Fuemmeler B F, Elkin T D, Mullins L L. Survivors of childhood brain tumors: behavioral, emotional, and social adjustment. Clin Psychol Rev. 2002; 22 547-585
- 14 Goldwein J W, Radcliffe J, Johnson J, Moshang T, Packer R J. et al . Updated results of a pilot study of low dose craniospinal irradiation plus chemotherapy for children under five with cerebellar primitive neuroectodermal tumors (medulloblastoma). Int J Radiat Oncol Biol Phys. 1996; 34 899-904
- 15 Grissemann H. Zürcher Lesetest ZLT. Bern; Huber 2000
- 16 Grotzer M A. Current outcome predictors in childhood primitive neuroectodermal tumors. J Pediatr Neurol. 2003; 1 75-82
-
17 Gurney J G, Smith M A, Bunin G R.
CNS and miscellaneous intracranial and intraspinal neoplasms. SEER Pediatric Monograph. http://seer.cancer.gov/Publications/: National Cancer Institute. 2000: 51-63 - 18 Hammill D, Pearson N, Voress J. Developmental Test of Visual Perception. DTVP‐2. Austin; pro-ed 1993
- 19 Hooper H. Hooper Visual Organization Test (VOT). Los Angeles; Western Psychological Services 1983
- 20 Hoppe-Hirsch E, Brunet L, Laroussinie F, Cinalli G, Pierre-Kahn A. et al . Intellectual outcome in children with malignant tumors of the posterior fossa: influence of the field of irradiation and quality of surgery. Childs Nerv Syst. 1995; 11 340-346
- 21 Jenkin D. The radiation treatment of medulloblastoma. J Neuro-Oncol. 1996; 29 45-54
- 22 Jenney M EM. 1998. Theoretical issues pertinent to measurement of quality of life. Med Pediatr Oncol. 1998; 31 (Suppl I) 42-45
- 23 Johnson D L, McCabe M A, Nicholson H S, Joseph A L, Getson P R. et al . Quality of long-term survival in young children with medulloblastoma. J Neurosurg. 1994; 80 1004-1010
- 24 Kaufman A, Kaufman N. Kaufman Assessment Battery for Children. K‐ABC. Deutschsprachige Fassung von P. Melchers und U. Preuss. 4. korrigierte und ergänzte Auflage. Frankfurt am Main; Swets & Zeitlinger 1994
- 25 Kennedy C R, Leyland K. Comparison of screening instruments for disability and emotional/behavioral disorders with a generic measure of health-related quality of life in survivors of childhood brain tumors. Int J Cancer. 1999; 83 (Suppl 12) 106-111
- 26 Kunert H, Derichs G, Irle E. Entwicklung von Aufmerksamkeitsfunktionen im Kindersalter: Ergebnisse einer vorläufigen Normierung computerunterstützten Testbatterie zur Aufmerksamkeitsprüfung (TAP) an 9- bis 12jährigen Kindern. Z Neuropsychol. 1996; 2 92-113
- 27 LeBaron S, Zeltzer P M, Zeltzer L K, Scott S E, Marlin A E. Assessment of quality of survival in children with medulloblastoma and cerebellar astrocytoma. Cancer. 1988; 62 1215-1222
- 28 Mulhern R K, Carpentieri S, Shema S, Stone P, Fairclough D. Factors associated with social and behavioral problems among children recently diagnosed with brain tumor. J Pediatr Psychol. 1993; 18 339-350
- 29 Mulhern R K, Kepner J L, Thomas P R, Armstrong F D, Friedman H S, Kun L E. Neuropsychologic functioning of survivors of childhood medulloblastoma randomized to receive conventional or reduced-dose craniospinal irradiation: a pediatric oncology group study. J Clin Oncol. 1998; 16 1723-1728
- 30 Noll R B, Ris M D, Davies W H, Bukowski W M, Koontz K. Social interactions between children with cancer or sickle cell disease and their peers: teacher ratings. J Dev Behav Pediatr. 1992; 13 187-193
- 31 Olshan J S, Gubernick J, Packer R, D'Angio G J, Goldwein J W. et al . The effects of adjuvant chemotherapy on growth in children with medulloblastoma. Cancer. 1992; 70 2013-2017
- 32 Packer R J, Sutton L N, Atkins T E, Radcliffe J, Bunin G R. et al . A prospective study of cognitive function in children receiving whole-brain radiotherapy and chemotherapy: 2-year results. J Neurosurg. 1989; 70 707-713
- 33 Pendley J S, Dahlquist L M, Dreyer Z. Body image and psychosocial adjustment in adolescent cancer survivors. J Pediatr Psychol. 1997; 22 29-43
- 34 Radcliffe J, Packer R J, Atkins T E, Bunin G R, Schut L. et al . Three- and four-year cognitive outcome in children with noncortical brain tumors treated with whole-brain radiotherapy. Ann Neurol. 1992; 32 551-554
- 35 Regard M, Strauss E, Knapp P. Children's production on verbal and non-verbal fluency tasks. Percept Mot Skills. 1982; 55 839-844
- 36 Rorke L B, Trojanowski J Q, Lee V M, Zimmerman R A, Sutton L N. et al . Primitive neuroectodermal tumors of the central nervous system. Brain Pathol. 1997; 7 765-784
- 37 Schellig D, Hättig H. Die Bestimmung der visuellen Merkspanne mit dem Block-Board. Z Neuropsychol. 1993; 2 104-112
-
38 Schipper H, Clinch J, Olweny C.
Quality of life studies: definitions and conceptual frameworks. Spilliker B Quality of Life and Pharmacoeconomics in Clinical Trials. Philadelphia; Lippincott Williams & Wilkins Publishers 1996 - 39 Schweisthal B. Die Leistungen von 7- bis 15jährigen Kindern im verbalen Lern- und Merkfähigkeits-Test (VLMT). Z Neuropsychol. 1997; 2 129-139
- 40 Silber J H, Radcliffe J, Peckham V, Perilongo G, Kishnani P. et al . Whole-brain irradiation and decline in intelligence: the influence of dose and age on IQ score. J Clin Oncol. 1992; 10 1390-1396
- 41 Silverman C L, Palkes H, Talent B, Kovnar E, Clouse J W, Thomas P RM. Late effects of radiotherapy of patients with cerebellar medulloblastoma. Cancer. 1984; 54 825-829
- 42 Spreen O, Strauss E. A compendium of neuropsychological tests. New York; Oxford University Press 1991
- 43 Steinhausen H C, Winkler-Metzke C, Kannenberg R. Handbuch: Elternfragebogen über das Verhalten von Kindern und Jugendlichen. Die Zürcher Ergebnisse zur deutschen Fassung der Child Behavior Checklist (CBCL). Zurich; Department of Child and Adolescent Psychiatry, University of Zurich 1996
- 44 Steinhausen H C, Winkler-Metzke C, Kannenberg R. Handbuch. Fragebogen für Jugendliche. Zürcher Ergebnisse zur deutschen Fassung des Youth Self Report (YSR). Zurich; Department of Child and Adolescent Psychiatry, University of Zurich 1999
- 45 Steinhausen H C, Winkler Metzke C. Youth self report of behavioral and emotional problems in a Swiss epidemiological study. J Youth Adolesc. 1998; 27 425-441
- 46 Tewes U. Hamburg-Wechsler-Intelligenztest für Erwachsene - Revision 1991. HAWIE‐R. 2. korrigierte Auflage. Bern; Verlag Hans Huber 1994
- 47 Tewes U. Hamburg-Wechsler-Intelligenztest für Kinder III. HAWIK‐III. Bern; Verlag Hans Huber 1999
- 48 Theunissen N C, Vogels T G, Koopman H M, Verrips G H, Zwinderman K A. et al . The proxy problem: child report versus parent report in health-related quality of life research. Qual Life Res. 1998; 7 387-397
- 49 Vannatta K, Gartstein M A, Short A, Noll R B. A controlled study of peer relationships of children surviving brain tumors: teacher, peer, and self ratings. J Pediatr Psychol. 1998; 23 279-287
- 50 Varni J W, Burwinkle T M, Katz E R, Meeske K, Dickinson P. The PedsQL in pediatric cancer: reliability and validity of the Pediatric Quality of Life Inventory Generic Core Scales, Multidimensional Fatigue Scale, and Cancer Module. Cancer. 2002; 94 2090-2106
- 51 Varni J W, Seid M, Kurtin P S. PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient populations. Med Care. 2001; 39 800-812
- 52 Varni J W, Seid M, Rode C A. The PedsQL: measurement model for the pediatric quality of life inventory. Med Care. 1999; 37 126-139
- 53 Wehrli A. Neuropsychologische Untersuchung im Kindesalter. Zürich; Universität Zürich 1980
- 54 WHO .Constitution of the World Health Organization. Geneva; WHO 1948
- 55 Zimmermann P, Fimm B. Testbatterie zur Aufmerksamkeitsprüfung (TAP). Herzogenrath; Psytest 1993
PD Dr. med. Michael Grotzer
University Children's Hospital
Steinwiesstrasse 75
8032 Zurich
Switzerland
Email: Michael.Grotzer@kispi.unizh.ch