Ependymoma in childhood: prognostic factors, extent of surgery, and adjuvant therapy

Restricted access

Object. The aim of this study was to investigate the effect of patient-related factors, extent of surgery, and adjuvant therapy on survival in children presenting with intracranial ependymoma.

Methods. Between 1980 and 1999, 83 children (mean age 36 months) underwent surgery for intracranial ependymomas. Complete resection, verified on postoperative computerized tomography scans, was achieved in 73%. Adjuvant therapy modalities have changed over the years: before 1990 all patients received radiotherapy, whereas after 1990 the children younger than 3 years of age and later those younger than 5 years of age were treated first with chemotherapy and received radiotherapy only after their first tumor recurrence. The follow-up period averaged 70 x 49 months.

Conclusions. Overall survival, intraoperative deaths excluded, was 73 ± 11% and 51 ± 14% at 5 and 10 years, respectively. The event-free survival rate at 5 and 10 years was 48 ± 12% and 46 ± 12%, respectively. Most of the events were local recurrences. Despite multiple reinterventions, the overall survival rate in this group dropped to 14%. On univariate analysis, the only significant prognostic factors were complete resection and radiotherapy. Both of these factors combined increased the 5- and 10-year survival rates to 93 and 75%, respectively. Age of the patients was not a statistically independent prognostic factor. The patients in the chemotherapy group did not fare as well as those in the radiotherapy group. A subgroup (36%) within the chemotherapy group, however, survived tumor free after a mean follow-up period of 67 months. It is not clear whether this subgroup either responded well to chemotherapy or needed no adjuvant therapy. Further research is warranted to answer this question.

Article Information

Contributor Notes

Address reprint requests to: Alain Pierre-Kahn, M.D., Department of Pediatric Neurosurgery, Groupe Hospitalier Necker-Enfants Malades, 149, rue de Sèvres, 75743 Paris Cedex 15, France. email: alain.kahn@nck.ap-hop-paris.fr.
Headings
References
  • 1.

    Awaad YMAllen JCMiller DCet al: Deferring adjuvant therapy for totally resected intracranial ependymoma. Pediatr Neurol 14:2162191996Awaad YM Allen JC Miller DC et al: Deferring adjuvant therapy for totally resected intracranial ependymoma. Pediatr Neurol 14:216–219 1996

    • Search Google Scholar
    • Export Citation
  • 2.

    Bennetto LForeman NHardin Bet al: Ki-67 immunolabeling index is a prognostic indicator in childhood posterior fossa ependymomas. Neuropathol Appl Neurobiol 24:4344401998Bennetto L Foreman N Hardin B et al: Ki-67 immunolabeling index is a prognostic indicator in childhood posterior fossa ependymomas. Neuropathol Appl Neurobiol 24:434–440 1998

    • Search Google Scholar
    • Export Citation
  • 3.

    Bouffet EPerilongo GCanete Aet al: Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation. Med Pediatr Oncol 30:3193311998Bouffet E Perilongo G Canete A et al: Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation. Med Pediatr Oncol 30:319–331 1998

    • Search Google Scholar
    • Export Citation
  • 4.

    Chiu JKWoo SyAter Jet al: Intracranial ependymoma in children: analysis of prognostic factors. J Neurooncol 13:2832901992Chiu JK Woo Sy Ater J et al: Intracranial ependymoma in children: analysis of prognostic factors. J Neurooncol 13:283–290 1992

    • Search Google Scholar
    • Export Citation
  • 5.

    Dohrmann GJFarwell JRFlannery JT: Ependymomas and ependymoblastomas in children. J Neurosurg 45:2732831976Dohrmann GJ Farwell JR Flannery JT: Ependymomas and ependymoblastomas in children. J Neurosurg 45:273–283 1976

    • Search Google Scholar
    • Export Citation
  • 6.

    Duffner PKHorowitz MEKrischer JPet al: Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 328:172517311993Duffner PK Horowitz ME Krischer JP et al: Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 328:1725–1731 1993

    • Search Google Scholar
    • Export Citation
  • 7.

    Evans AEAnderson JRLefkowitz-Boudreaux IBet al: Adjuvant chemotherapy of childhood posterior fossa ependymoma: cranio-spinal irradiation with or without adjuvant CCNU, vincristine and prednisone: a Childrens Cancer Group study. Med Pediatr Oncol 27:8141996Evans AE Anderson JR Lefkowitz-Boudreaux IB et al: Adjuvant chemotherapy of childhood posterior fossa ependymoma: cranio-spinal irradiation with or without adjuvant CCNU vincristine and prednisone: a Childrens Cancer Group study. Med Pediatr Oncol 27:8–14 1996

    • Search Google Scholar
    • Export Citation
  • 8.

    Figarella-Branger DCivatte MBouvier-Labit Cet al: Prognostic factors in intracranial ependymomas in children. J Neurosurg 93:6056132000Figarella-Branger D Civatte M Bouvier-Labit C et al: Prognostic factors in intracranial ependymomas in children. J Neurosurg 93:605–613 2000

    • Search Google Scholar
    • Export Citation
  • 9.

    Finlay JLBoyett JMYates AJet al: Randomized phase III trial in childhood high-grade astrocytoma comparing vincristine, lomustine, and prednisone with the eight-drugs-in-1-day regimen. Childrens Cancer Group. J Clin Oncol 13:1121231995Finlay JL Boyett JM Yates AJ et al: Randomized phase III trial in childhood high-grade astrocytoma comparing vincristine lomustine and prednisone with the eight-drugs-in-1-day regimen. Childrens Cancer Group. J Clin Oncol 13:112–123 1995

    • Search Google Scholar
    • Export Citation
  • 10.

    Foreman NKLove SGill SSet al: Second-look surgery for incompletely resected fourth ventricle ependymomas: technical case report. Neurosurgery 40:8568601997Foreman NK Love S Gill SS et al: Second-look surgery for incompletely resected fourth ventricle ependymomas: technical case report. Neurosurgery 40:856–860 1997

    • Search Google Scholar
    • Export Citation
  • 11.

    Foreman NKLove SThorne R: Intracranial ependymomas: analysis of prognostic factors in a population-based series. Pediatr Neurosurg 24:1191251996Foreman NK Love S Thorne R: Intracranial ependymomas: analysis of prognostic factors in a population-based series. Pediatr Neurosurg 24:119–125 1996

    • Search Google Scholar
    • Export Citation
  • 12.

    Friedman HSKrischer JPBurger Pet al: Treatment of children with progressive or recurrent brain tumors with carboplatin of iproplatin: a Pediatric Oncology Group randomized phase II study. J Clin Oncol 10:2492561992Friedman HS Krischer JP Burger P et al: Treatment of children with progressive or recurrent brain tumors with carboplatin of iproplatin: a Pediatric Oncology Group randomized phase II study. J Clin Oncol 10:249–256 1992

    • Search Google Scholar
    • Export Citation
  • 13.

    Gerszten PCPollack IFMartinez AJet al: Intracranial ependymomas of childhood. Lack of correlation of histopathology and clinical outcome. Pathol Res Pract 192:5155221996Gerszten PC Pollack IF Martinez AJ et al: Intracranial ependymomas of childhood. Lack of correlation of histopathology and clinical outcome. Pathol Res Pract 192:515–522 1996

    • Search Google Scholar
    • Export Citation
  • 14.

    Goldwein JWGlauser TAPacker RJet al: Recurrent intracranial ependymomas in children. Survival, patterns of failure, and prognostic factors. Cancer 66:5575631990Goldwein JW Glauser TA Packer RJ et al: Recurrent intracranial ependymomas in children. Survival patterns of failure and prognostic factors. Cancer 66:557–563 1990

    • Search Google Scholar
    • Export Citation
  • 15.

    Grill JKalifa CDoz Fet al: A high-dose busulfan-thiotepa combination followed by autologous bone marrow transplantation in childhood recurrent ependymoma. A phase-II study. Pediatr Neurosurg 25:7121996Grill J Kalifa C Doz F et al: A high-dose busulfan-thiotepa combination followed by autologous bone marrow transplantation in childhood recurrent ependymoma. A phase-II study. Pediatr Neurosurg 25:7–12 1996

    • Search Google Scholar
    • Export Citation
  • 16.

    Grill JLe Deley MCGambarelli Det al: Postoperative chemotherapy without irradiation for ependymoma in children under 5 years of age: a multicenter trial of the French Society of Pediatric Oncology. J Clin Oncol 19:128812962001Grill J Le Deley MC Gambarelli D et al: Postoperative chemotherapy without irradiation for ependymoma in children under 5 years of age: a multicenter trial of the French Society of Pediatric Oncology. J Clin Oncol 19:1288–1296 2001

    • Search Google Scholar
    • Export Citation
  • 17.

    Hamilton RLPollack IF: The molecular biology of ependymomas. Brain Pathol 7:8078221997Hamilton RL Pollack IF: The molecular biology of ependymomas. Brain Pathol 7:807–822 1997

    • Search Google Scholar
    • Export Citation
  • 18.

    Healey EABarnes PDKupsky WJet al: The prognostic significance of postoperative residual tumor in ependymoma. Neurosurgery 28:6666721991Healey EA Barnes PD Kupsky WJ et al: The prognostic significance of postoperative residual tumor in ependymoma. Neurosurgery 28:666–672 1991

    • Search Google Scholar
    • Export Citation
  • 19.

    Horn BHeideman RGeyer Ret al: A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol 21:2012111999Horn B Heideman R Geyer R et al: A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol 21:201–211 1999

    • Search Google Scholar
    • Export Citation
  • 20.

    Hukin JEpstein FLefton Det al: Treatment of intracranial ependymoma by surgery alone. Pediatr Neurosurg 29:40451998Hukin J Epstein F Lefton D et al: Treatment of intracranial ependymoma by surgery alone. Pediatr Neurosurg 29:40–45 1998

    • Search Google Scholar
    • Export Citation
  • 21.

    Ikezaki KMatsushima TInoue Tet al: Correlation of microanatomical localization with postoperative survival in posterior fossa ependymomas. Neurosurgery 32:38441993Ikezaki K Matsushima T Inoue T et al: Correlation of microanatomical localization with postoperative survival in posterior fossa ependymomas. Neurosurgery 32:38–44 1993

    • Search Google Scholar
    • Export Citation
  • 22.

    Kleihues PBurger PCScheithauer BW: The new WHO classification of brain tumours. Brain Pathol 3:2552681993Kleihues P Burger PC Scheithauer BW: The new WHO classification of brain tumours. Brain Pathol 3:255–268 1993

    • Search Google Scholar
    • Export Citation
  • 23.

    Lyons MKKelly PJ: Posterior fossa ependymomas: report of 30 cases and review of the literature. Neurosurgery 28:6596651991Lyons MK Kelly PJ: Posterior fossa ependymomas: report of 30 cases and review of the literature. Neurosurgery 28:659–665 1991

    • Search Google Scholar
    • Export Citation
  • 24.

    Mason WPGoldman SYates AJet al: Survival following intensive chemotherapy with bone marrow reconstitution for children with recurrent intracranial ependymoma—a report of the Children's Cancer Group. J Neurooncol 37:1351431998Mason WP Goldman S Yates AJ et al: Survival following intensive chemotherapy with bone marrow reconstitution for children with recurrent intracranial ependymoma—a report of the Children's Cancer Group. J Neurooncol 37:135–143 1998

    • Search Google Scholar
    • Export Citation
  • 25.

    Nazar GBHoffman HJBecker LEet al: Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg 72:4084171990Nazar GB Hoffman HJ Becker LE et al: Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg 72:408–417 1990

    • Search Google Scholar
    • Export Citation
  • 26.

    Needle MNGoldwein JWGrass Jet al: Adjuvant chemotherapy for the treatment of intracranial ependymoma of childhood. Cancer 80:3413471997Needle MN Goldwein JW Grass J et al: Adjuvant chemotherapy for the treatment of intracranial ependymoma of childhood. Cancer 80:341–347 1997

    • Search Google Scholar
    • Export Citation
  • 27.

    Papadopoulos DPGiri SEvans RG: Prognostic factors and management of intracranial ependymomas. Anticancer Res 10:6896921990Papadopoulos DP Giri S Evans RG: Prognostic factors and management of intracranial ependymomas. Anticancer Res 10:689–692 1990

    • Search Google Scholar
    • Export Citation
  • 28.

    Perilongo GMassimino MSotti Get al: Analyses of prognostic factors in a retrospective review of 92 children with ependymoma: Italian Pediatric Neuro-Oncology Group. Med Pediatr Oncol 29:79851997Perilongo G Massimino M Sotti G et al: 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

    • Search Google Scholar
    • Export Citation
  • 29.

    Pollack IFGerszten PCMartinez AJet al: Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 37:6556671995Pollack IF Gerszten PC Martinez AJ et al: Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 37:655–667 1995

    • Search Google Scholar
    • Export Citation
  • 30.

    Prayson RA: Clinicopathologic study of 61 patients with ependymoma including MIB-1 immunohistochemistry. Ann Diagn Pathol 3:11181999Prayson RA: Clinicopathologic study of 61 patients with ependymoma including MIB-1 immunohistochemistry. Ann Diagn Pathol 3:11–18 1999

    • Search Google Scholar
    • Export Citation
  • 31.

    Ritter AMHess KRMcLendon REet al: Ependymomas: MIB-I proliferation index and survival. J Neurooncol 40:51571998Ritter AM Hess KR McLendon RE et al: Ependymomas: MIB-I proliferation index and survival. J Neurooncol 40:51–57 1998

    • Search Google Scholar
    • Export Citation
  • 32.

    Robertson PLZeltzer PMBoyett JMet al: Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children's Cancer Group. J Neurosurg 88:6957031998Robertson PL Zeltzer PM Boyett JM et al: 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

    • Search Google Scholar
    • Export Citation
  • 33.

    Ross GWRubinstein LJ: Lack of histopathological correlation of malignant ependymomas with postoperative survival. J Neurosurg 70:31361989Ross GW Rubinstein LJ: Lack of histopathological correlation of malignant ependymomas with postoperative survival. J Neurosurg 70:31–36 1989

    • Search Google Scholar
    • Export Citation
  • 34.

    Rousseau PHabrand JLSarrazin Det al: Treatment of intracranial ependymomas of children: review of a 15-year experience. Int J Radiat Oncol Biol Phys 28:3813861994Rousseau P Habrand JL Sarrazin D et al: Treatment of intracranial ependymomas of children: review of a 15-year experience. Int J Radiat Oncol Biol Phys 28:381–386 1994

    • Search Google Scholar
    • Export Citation
  • 35.

    Sala FTalacchi AMazza Cet al: Prognostic factors in childhood intracranial ependymomas: the role of age and tumor location. Pediatr Neurosurg 28:1351421998Sala F Talacchi A Mazza C et al: Prognostic factors in childhood intracranial ependymomas: the role of age and tumor location. Pediatr Neurosurg 28:135–142 1998

    • Search Google Scholar
    • Export Citation
  • 36.

    Schiffer DChio ACravioto Het al: Ependymoma: internal correlations among pathological signs: the anaplastic variant. Neurosurgery 29:2062101991Schiffer D Chio A Cravioto H et al: Ependymoma: internal correlations among pathological signs: the anaplastic variant. Neurosurgery 29:206–210 1991

    • Search Google Scholar
    • Export Citation
  • 37.

    Schiffer DChio AGiordana MTet al: Histologic prognostic factors in ependymoma. Childs Nerv Syst 7:1771821991Schiffer D Chio A Giordana MT et al: Histologic prognostic factors in ependymoma. Childs Nerv Syst 7:177–182 1991

    • Search Google Scholar
    • Export Citation
  • 38.

    Schild SENisi KScheithauer BWet al: The results of radiotherapy for ependymomas: the Mayo Clinic experience. Int J Radiat Oncol Biol Phys 42:9539581998Schild SE Nisi K Scheithauer BW et al: The results of radiotherapy for ependymomas: the Mayo Clinic experience. Int J Radiat Oncol Biol Phys 42:953–958 1998

    • Search Google Scholar
    • Export Citation
  • 39.

    Schwartz THKim SGlick RSet al: Supratentorial ependymomas in adult patients. Neurosurgery 44:7217311999Schwartz TH Kim S Glick RS et al: Supratentorial ependymomas in adult patients. Neurosurgery 44:721–731 1999

    • Search Google Scholar
    • Export Citation
  • 40.

    Shaw EGEvans RGScheithauer BWet al: Postopertive radiotherapy of intracranial ependymoma in pediatric and adult patients. Int J Radiat Oncol Phys 13:145714621987Shaw EG Evans RG Scheithauer BW et al: Postopertive radiotherapy of intracranial ependymoma in pediatric and adult patients. Int J Radiat Oncol Phys 13:1457–1462 1987

    • Search Google Scholar
    • Export Citation
  • 41.

    Vanuytsel LBrada M: The role of prophylactic spinal irradiation in localized intracranial ependymoma. Int J Radiat Oncol Biol Phys 21:8258301991Vanuytsel L Brada M: The role of prophylactic spinal irradiation in localized intracranial ependymoma. Int J Radiat Oncol Biol Phys 21:825–830 1991

    • Search Google Scholar
    • Export Citation
  • 42.

    von Haken MSWhite ECDaneshvar-Shyesther Let al: Molecular genetic analysis of chromosome arm 17p and chromosome arm 22q DNA sequences in sporadic pediatric ependymomas. Genes Chromosomes Cancer 17:37441996von Haken MS White EC Daneshvar-Shyesther L et al: Molecular genetic analysis of chromosome arm 17p and chromosome arm 22q DNA sequences in sporadic pediatric ependymomas. Genes Chromosomes Cancer 17:37–44 1996

    • Search Google Scholar
    • Export Citation
TrendMD
Cited By
Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 190 82 8
Full Text Views 173 13 0
PDF Downloads 143 14 0
EPUB Downloads 0 0 0
PubMed
Google Scholar