Skip to main content
SpringerLink
Log in

Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report

  • Clinical study - patient studies
  • Published:
Journal of Neuro-Oncology Aims and scope Submit manuscript

Abstract

Introduction Supratentorial primitive neuroectodermal tumors (SPNET) are rare tumors accounting for only 2.5% of childhood brain tumors. The purpose of this study was to describe the range of treatment regimens used to treat pediatric SPNET in Canada and to identify prognostic factors for overall survival in this population. Methods This study was a retrospective clinical analysis of SPNET patients treated over the last 10 years in Canada. A questionnaire was developed and distributed to all institutions in Canada who treat pediatric patients. Data were collected for patients <19 years of age who were diagnosed and treated for SPNET between 1995 and 2005. Results Data were obtained for 48 eligible patients. The stages of patients for whom complete data were provided were 80, 3, and 16% for metastatic stage M0, M1, and M2/3, respectively. The best responses to therapy included complete response in 44%, partial response in 8%, still on therapy in 2%, progressive disease in 31%, toxic death in 2%, and no therapy given in 12%. The 4-year survival was 37.7 ± 7.6%. The factors associated with an increase in survival were the use of radiation therapy and chemotherapy, and age >2 years. Overall survival was not affected by metastatic disease at diagnosis, tumor site, or degree of initial resection. Conclusions Survival is poor in SPNET patients but highest in those who received chemotherapy and radiation therapy. Further studies are needed to improve the survival of these patients.

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

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Gaffney CC, Sloane JP, Bradley NJ, Bloom HJG (1985) Primitive neuroectodermal tumours of the cerebrum. J Neurooncol 3:23–33

    Article  PubMed  CAS  Google Scholar 

  2. Albright AL, Wisoff JH, Zeltzer P et al (1995) Prognostic factors in children with supratentorial (nonpineal) primitive neuroectodermal tumors. Pediatr Neurosurg 22:1–7

    PubMed  CAS  Google Scholar 

  3. Rorke LB, Trojanowski JQ, Lee VM et al (1997) Primitive neuroectodermal tumors of the central nervous system. Brain Pathol 2:765–784

    Google Scholar 

  4. Jakacki RI (1999) Pineal and nonpineal supratentorial primitive neuroectodermal tumors. Child Nerv Syst 15(10):586–591

    Article  CAS  Google Scholar 

  5. Li MH, Bouffet E, Hawkins CE, Squire JA, Huang A (2005) Molecular genetics of supratentorial primitive neuroectodermal tumors and pineoblastoma. Neurosurg Focus 19:E3

    PubMed  Google Scholar 

  6. Dirks PB, Harris L, Hoffman HJ, Humphreys RP, Drake JM, Rutka JT (1996) Supratentorial primitive neuroectodermal tumors in children. J Neurooncol 29:75–84

    Article  PubMed  CAS  Google Scholar 

  7. Cohen BH, Zeltzer PM, Boyett JM et al (1995) Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: a childrens’ cancer group randomized trial. J Clin Oncol 13:1687–1696

    PubMed  CAS  Google Scholar 

  8. Albright AL, Wisoff JH, Zeltzer P et al (1995) Prognostic factors in children with supratentorial (nonpineal) primitive neuroectodermal tumors. Pediatr Neurosurg 22:1–7

    Article  PubMed  CAS  Google Scholar 

  9. Jakacki R (1999) Pineal and nonpineal supratentorial primitive neuroectodermal tumors. Child Nerv Syst 15:586–591

    Article  CAS  Google Scholar 

  10. Yang HJ, Nam DH, Wang KC, Kim YM, Chi JG, Cho BK (1999) Supratentorial primitive neuroectodermal tumor in children: clinical features, treatment outcome and prognostic factors. Child Nerv Syst 15:377–383

    Article  CAS  Google Scholar 

  11. Paulino AC, Melian E (1999) Medulloblastoma and supratentorial primitive neuroectodermal tumors, an institutional experience. Cancer 86:142–148

    Article  PubMed  CAS  Google Scholar 

  12. Reddy AT, Janss AJ, Phillips PC, Weiss HL, Packer RJ (2000) Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation and chemotherapy. Cancer 88:2189–2193

    Article  PubMed  CAS  Google Scholar 

  13. Geyer JR, Sposto R, Jennings M et al (2005) Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: A report from the Children’s Cancer Group. J Clin Oncol 23:7621–7631

    Article  PubMed  Google Scholar 

  14. Pizer BL, Weston CL, Robinson KJ et al (2006) Analysis of patients with supratentorial primitive neuro-ectodermal tumors entered into the SIOP/UKCCSG PNET 3 study. Eur J Cancer 42:1120–1128

    Article  PubMed  Google Scholar 

  15. Timmermann B, Kortmann RD, Kuhl J et al (2002) Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective German brain tumor trials HIT 88/89 and 91. J Clin Oncol 20:842–849

    Article  PubMed  Google Scholar 

  16. Jakacki RI, Zeltzer PM, Boyett JM et al (1995) Survival and prognostic factors following radiation and/or chemotherapy for primitive neuroectodermal tumors of the pineal region in infants and children: a report of the childrens cancer group. J Clin Oncol 13:1377–1383

    PubMed  CAS  Google Scholar 

  17. Marec-Berard B, Jouvet A, Thiesse P, Kalifa C, Doz F, Frappaz D (2002) Supratentorial embryonal tumors in children under 5 years of age: a SFOP study of treatment with postoperative chemotherapy alone. Med Pediatr Oncol 38:83–90

    Article  PubMed  CAS  Google Scholar 

  18. Timmermann B, Kortmann RD, Kuhl J et al (2006) Role of radiotherapy in supratentorial primitive neuroectodermal tumor in young children: Results of the German HIT-SKK87 and HIT-SKK92 trials. J Clin Oncol 24:1554–1560

    Article  PubMed  Google Scholar 

  19. Duchatelier S, Wolf SM (2001) Diagnostic principles. In: Keating RF, Goodrich JT, Packer RJ (eds) Tumors of the pediatric central nervous system. Thieme, New York, pp 22–26

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Hematology/Oncology, Children’s Hospital of Eastern Ontario, 401 Smyth Road, Ottawa, ON, Canada, K1H 8L1

    Donna L. Johnston

  2. Division of Neurology, Children’s Hospital of Eastern Ontario, 401 Smyth Road, Ottawa, ON, Canada, K1H 8L1

    Daniel L. Keene

  3. The Hospital for Sick Children, 555 University Ave, Toronto, ON, Canada, M5G 1X8

    Lucie Lafay-Cousin, Lillian Sung, Annie Huang & Eric Bouffet

  4. B.C. Children’s Hospital, 4480 Oak St, Vancouver, BC, Canada, V6H 3V4

    Paul Steinbok

  5. Montreal Children’s Hospital, 2300 rue Tupper, Montreal, QC, Canada, H3H 1P3

    Anne-Sophie Carret

  6. IWK Health Center, 5980 University Ave, Halifax, NS, Canada, B3K 6R8

    Bruce Crooks

  7. Departments of Oncology and Pediatrics, Faculty of Medicine, University of Calgary, 2888 Shaganappi Trail NW, Calgary, AB, Canada, T3B 6A8

    Douglas Strother

  8. Stollery Children’s Hospital, 11402 University Ave, Edmonton, AB, Canada, T6G 2J3

    Beverly Wilson

  9. Hamilton Health Sciences, 1200 Main St W, Hamilton, ON, Canada, L8S 4J9

    Isaac Odame

  10. Winnipeg Children’s Hospital, 840 Sherbrook St, Winnipeg, MB, Canada, R3A 1S1

    David D. Eisenstat

  11. Children’s Health Foundation, 20 Campus Dr, Saskatoon, SK, Canada, S7N 4H4

    Chris Mpofu

  12. Children’s Hospital of Western Ontario, 800 Commissioners Rd E, London, ON, Canada, N6A 4G5

    Shayna Zelcer

Authors
  1. Donna L. Johnston
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Daniel L. Keene
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Lucie Lafay-Cousin
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Paul Steinbok
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Lillian Sung
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Anne-Sophie Carret
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Bruce Crooks
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Douglas Strother
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Beverly Wilson
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Isaac Odame
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. David D. Eisenstat
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Chris Mpofu
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Shayna Zelcer
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Annie Huang
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Eric Bouffet
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Donna L. Johnston.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Johnston, D.L., Keene, D.L., Lafay-Cousin, L. et al. Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report. J Neurooncol 86, 101–108 (2008). https://doi.org/10.1007/s11060-007-9440-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11060-007-9440-1

Keywords

Search

Navigation