Cent Eur Neurosurg 2011; 72(3): 127-132
DOI: 10.1055/s-0031-1273745
Original Article

© Georg Thieme Verlag KG Stuttgart · New York

Treatment Results in Patients with Intracranial Ependymomas

A. Boström1 , J. Boström1 , W. Hartmann2 , T. Pietsch3 , M. Feuss1 , M. von Lehe1 , M. Simon1
  • 1University Hospital of Bonn Medical Center, Department of Neurosurgery, Bonn, Germany
  • 2University Hospital of Bonn Medical Center, Department of Pathology, Bonn, Germany
  • 3University Hospital of Bonn Medical Center, Department of Neuropathology, Bonn, Germany
Further Information

Publication History

Publication Date:
31 May 2011 (online)

Abstract

Objective: To evaluate the treatment outcomes of patients with intracranial ependymomas.

Methods: Between 1988–2007, 27 consecutive patients, with 9 patients (33%) aged under 16 years, were treated at our institution for an intracranial ependymoma. Pertinent clinical data were retrieved from the patients’ charts. The histopathological findings in 25 cases were reviewed using the 2007 World Health Organization (WHO) classification system. Median follow-up was 84 months.

Results: Infratentorial tumors were diagnosed in 22, and supratentorial tumors in 5 patients. Histopathological findings were ependymoma WHO grade II (E II) in 14 patients, and anaplastic ependymoma WHO grade III (AE III) in 13 patients. A complete tumor resection was achieved with the first operation in 20 cases (74%). Primary adjuvant therapy consisted of chemotherapy alone in a 17-month-old child with an incompletely resected posterior fossa AE III, radiation therapy alone in 4 cases, and combined radiation therapy and chemotherapy in 7 cases. Tumor recurrence was seen in 10 cases (37%), including 5 patients with an E II and 5 patients with an AE III. The 5-year progression-free survival (PFS) was 74% and 67% for E II and AE III, respectively. The 5-year PFS was 80% following a complete resection, and 56% in patients with a residual tumor.

Conclusions: Surgery alone, as the primary treatment, achieves a good outcome in most patients with E II. Good results can be achieved with surgery and adjuvant local radiotherapy in patients with AE III.

References

  • 1 Kleihues P, Cavence W. Pathology and genetics of tumours of the nervous system. Lyon: IARC Press; 2000: 72-77
  • 2 Combs SE, Thilmann C, Debus J. et al . Local radiotherapeutic management of ependymomas with fractionated stereotactic radiotherapy (FSRT).  BMC Cancer. 2006;  6 222
  • 3 Tomita T, McLone DG, Das L. et al . Benign ependymomas of the posterior fossa in childhood.  Pediatr Neurosci. 1988;  14 277-285
  • 4 Louis DN, Ohgaki H, Wiestler OD. et al .WHO Classification of tumours of the central nervous system. 4th ed. Lyon: International Agency for Research on Cancer (IARC); 2007: 309
  • 5 Lednicky JA, Garcea RL, Bergsagel DJ. et al . Natural simian virus 40 strains are present in human choroid plexus and ependymoma tumors.  Virology. 1995;  212 710-717
  • 6 Weggen S, Bayer TA, von Deimling A. et al . Low frequency of SV40, JC and BK polyomavirus sequences in human medulloblastomas, meningiomas and ependymomas.  Brain Pathol. 2000;  10 85-92
  • 7 Hamilton RL, Pollack IF. The molecular biology of ependymomas.  Brain Pathol. 1997;  7 807-822
  • 8 Jeuken JW, Sprenger SH, Gilhuis J. et al . Correlation between localization, age, and chromosomal imbalances in ependymal tumours as detected by CGH.  J Pathol. 2002;  197 238-244
  • 9 Rickert CH, Paulus W. Comparative genomic hybridization in central and peripheral nervous system tumors of childhood and adolescence.  J Neuropathol Exp Neurol. 2004;  63 399-417
  • 10 Karnofsky DA, Burchenal JH. The Clinical Evaluation of Chemotherapeutic Agents in Cancer Columbia Univ Press; 1949: 196
  • 11 Reni M, Brandes AA, Vavassori V. et al . A multicenter study of the prognosis and treatment of adult brain ependymal tumors.  Cancer. 2004;  100 1221-1229
  • 12 Kawabata Y, Takahashi JA, Arakawa Y. et al . Long-term outcome in patients harboring intracranial ependymoma.  J Neurosurg. 2005;  103 31-37
  • 13 Mansur DB, Perry A, Rajaram V. et al . Postoperative radiation therapy for grade II and III intracranial ependymoma.  Int J Radiat Oncol Biol Phys. 2005;  61 387-391
  • 14 McCormick PC, Torres R, Post KD. et al . Intramedullary ependymoma of the spinal cord.  J Neurosurg. 1990;  72 523-532
  • 15 Roncaroli F, Consales A, Fioravanti A. et al . Supratentorial cortical ependymoma: report of three cases.  Neurosurgery. 2005;  57 E192 ; discussion E192
  • 16 Figarella-Branger D, Civatte M, Bouvier-Labit C. et al . Prognostic factors in intracranial ependymomas in children.  J Neurosurg. 2000;  93 605-613
  • 17 Lyons MK, Kelly PJ. Posterior fossa ependymomas: report of 30 cases and review of the literature.  Neurosurgery. 1991;  28 659-664 ; discussion 664–665
  • 18 Spagnoli D, Tomei G, Ceccarelli G. et al . Combined treatment of fourth ventricle ependymomas: report of 26 cases.  Surg Neurol. 2000;  54 19-26 ; discussion 26
  • 19 Korshunov A, Golanov A, Sycheva R. et al . The histologic grade is a main prognostic factor for patients with intracranial ependymomas treated in the microneurosurgical era: an analysis of 258 patients.  Cancer. 2004;  100 1230-1237
  • 20 Metellus P, Barrie M, Figarella-Branger D. et al . Multicentric French study on adult intracranial ependymomas: prognostic factors analysis and therapeutic considerations from a cohort of 152 patients.  Brain. 2007;  130 1338-1349
  • 21 Vanuytsel L, Brada M. The role of prophylactic spinal irradiation in localized intracranial ependymoma.  Int J Radiat Oncol Biol Phys. 1991;  21 825-830
  • 22 Ernestus RI, Schroder R, Stutzer H. et al . The clinical and prognostic relevance of grading in intracranial ependymomas.  Br J Neurosurg. 1997;  11 421-428
  • 23 Oya N, Shibamoto Y, Nagata Y. et al . Postoperative radiotherapy for intracranial ependymoma: analysis of prognostic factors and patterns of failure.  J Neurooncol. 2002;  56 87-94
  • 24 Rogers L, Pueschel J, Spetzler R. et al . Is gross-total resection sufficient treatment for posterior fossa ependymomas?.  J Neurosurg. 2005;  102 629-636
  • 25 Palma L, Celli P, Mariottini A. et al . The importance of surgery in supratentorial ependymomas. Long-term survival in a series of 23 cases.  Childs Nerv Syst. 2000;  16 170-175
  • 26 Awaad YM, Allen JC, Miller DC. et al . Deferring adjuvant therapy for totally resected intracranial ependymoma.  Pediatr Neurol. 1996;  14 216-219
  • 27 Lansky SB, List MA, Lansky LL. et al . The measurement of performance in childhood cancer patients.  Cancer. 1987;  60 1651-1656
  • 28 Schwartz TH, Kim S, Glick RS. et al . Supratentorial ependymomas in adult patients.  Neurosurgery. 1999;  44 721-731
  • 29 Schild SE, Nisi K, Scheithauer BW. et al . The results of radiotherapy for ependymomas: the Mayo Clinic experience.  Int J Radiat Oncol Biol Phys. 1998;  42 953-958
  • 30 Schiffer D, Chio A, Cravioto H. et al . Ependymoma: internal correlations among pathological signs: the anaplastic variant.  Neurosurgery. 1991;  29 206-210
  • 31 Rawlings 3rd  CE, Giangaspero F, Burger PC. et al . Ependymomas: a clinicopathologic study.  Surg Neurol. 1988;  29 271-281
  • 32 Grill 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. 2001;  19 1288-1296
  • 33 Vanuytsel LJ, Bessell EM, Ashley SE. et al . Intracranial ependymoma: long-term results of a policy of surgery and radiotherapy.  Int J Radiat Oncol Biol Phys. 1992;  23 313-319
  • 34 Robertson PL, Zeltzer PM, Boyett JM. et al . Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Childrenâs Cancer Group.  J Neurosurg. 1998;  88 695-703
  • 35 McLaughlin MP, Marcus Jr RB, Buatti JM. et al . Ependymoma: results, prognostic factors and treatment recommendations.  Int J Radiat Oncol Biol Phys. 1998;  40 845-850
  • 36 Guyotat J, Signorelli F, Desme S. et al . Intracranial ependymomas in adult patients: analyses of prognostic factors.  J Neurooncol. 2002;  60 255-268
  • 37 Gerszten PC, Pollack IF, Martinez AJ. et al . Intracranial ependymomas of childhood. Lack of correlation of histopathology and clinical outcome.  Pathol Res Pract. 1996;  192 515-522
  • 38 Sala F, Talacchi A, Mazza C. et al . Prognostic factors in childhood intracranial ependymomas: the role of age and tumor location.  Pediatr Neurosurg. 1998;  28 135-142
  • 39 Chiu JK, Woo SY, Ater J. et al . Intracranial ependymoma in children: analysis of prognostic factors.  J Neurooncol. 1992;  13 283-290
  • 40 Nazar GB, Hoffman HJ, Becker LE. et al . Infratentorial ependymomas in childhood: prognostic factors and treatment.  J Neurosurg. 1990;  72 408-417
  • 41 Pollack IF, Gerszten PC, Martinez AJ. et al . Intracranial ependymomas of childhood: long-term outcome and prognostic factors.  Neurosurgery. 1995;  37 655-666 ; discussion 666–667

Correspondence

Dr. A. Boström

University Hospital of Bonn

Medical Center

Department of Neurosurgery

Sigmund-Freud-Straße 25

53105 Bonn

Germany

Phone: + 49/0228/287 165 21

Fax: + 49/0228/287 165 73

Email: azize.bostroem@ukb.uni-bonn.de

    >