Chemotherapy for intracranial ependymoma in adults.
BACKGROUND
Ependymal tumors in adults are rare, accounting for less than 4% of primary tumors of the central nervous system in this age group. The low prevalence of intracranial ependymoma in adults limits the ability to perform clinical trials. Therefore, treatment decisions are based on small, mostly retrospective studies and the role of chemotherapy has remained unclear.


METHODS
We performed a retrospective study on 17 adult patients diagnosed with intracranial World Health Organisation grade II or III ependymoma, who were treated with chemotherapy at any time during the disease course. Benefit from chemotherapy was estimated by applying Macdonald criteria. Progression-free (PFS) and overall survival (OS) were calculated from start of chemotherapy, using the Kaplan-Meier method.


RESULTS
Eleven patients had supratentorial and 6 infratentorial tumors. Ten patients were treated with temozolomide (TMZ), 3 with procarbazine/lomustine/vincristine (PCV), 3 with platinum-based chemotherapy and 1 patient received epirubicin/ifosfamide. Response rates were as follows: TMZ 8/10 stable disease; PCV 3/3 stable disease; platinum-based chemotherapy 1/3 partial response; epirubicin/ifosfamide 1/1 complete response. PFS rates at 6, 12 and 24 months were 52.9, 35.3 and 23.5%. OS rates at 6, 12 and 24 months were 82.4, 82.4 and 70.1%. There was no indication for a favourable prognostic role of O(6)-methylguanyl-DNA-methyltransferase (MGMT) promoter methylation which was detected in 3/12 investigated tumors.


CONCLUSIONS
Survival outcomes in response to chemotherapy in adult intracranial ependymoma patients vary substantially, but individual patients may respond to any kind of chemotherapy. There were too few patients to compare survival data between chemotherapeutic subgroups.
Department of Neurology, University Hospital Zurich, Frauenklinikstrasse 26, 8091, Zurich, Switzerland. dorothee.gramatzki@usz.ch.
Dorothee
D
Gramatzki
Department of Neurology, University Hospital Zurich, Frauenklinikstrasse 26, 8091, Zurich, Switzerland.
Patrick
P
Roth
Department of Neuropathology, Heinrich-Heine-University, Moorenstrasse 5, 40225, Düsseldorf, Germany.|German Cancer Consortium (DKTK), partner site Essen/Düsseldorf, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69121, Heidelberg, Germany.
Jörg
J
Felsberg
Department of Oncology, University Hospital Zurich, Rämistrasse 100, 8091, Zurich, Switzerland.
Silvia
S
Hofer
Department of Neuropathology, University Hospital Zurich, Schmelzbergstrasse 12, 8091, Zurich, Switzerland.
Elisabeth J
EJ
Rushing
Institute for Medical Informatics, Statistics and Epidemiology, University of Leipzig, Härtelstrasse 16-18, 04107, Leipzig, Germany.
Bettina
B
Hentschel
Department of Neurosurgery, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, 20251, Hamburg, Germany.
Manfred
M
Westphal
Department of Neurosurgery, Technical University Dresden, Fetscherstrasse 74, 01307, Dresden, Germany.
Dietmar
D
Krex
Department of Neurosurgery, University of Bonn Medical School, Sigmund-Freud-Strasse 25, 53127, Bonn, Germany.
Matthias
M
Simon
Department of Neurosurgery, Ludwig Maximilian University Munich, Marchionistrasse 15, 81377, Munich, Germany.
Oliver
O
Schnell
Clinical Cooperation Unit Neurooncology, German Cancer Consortium (DKTK), German Cancer Research Center (DKFZ), and Neurology Clinic and National Center for Tumor Diseases, University Hospital Heidelberg, Im Neuenheimer Feld 672, 69120, Heidelberg, Germany.|Department of General Neurology, University Hospital Tübingen, Hoppe-Seyler-Strasse 3, 72076, Tübingen, Germany.
Wolfgang
W
Wick
German Cancer Consortium (DKTK), partner site Essen/Düsseldorf, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69121, Heidelberg, Germany.|Department of General Neurology, University Hospital Tübingen, Hoppe-Seyler-Strasse 3, 72076, Tübingen, Germany.
Guido
G
Reifenberger
Department of Neurology, University Hospital Zurich, Frauenklinikstrasse 26, 8091, Zurich, Switzerland.|Department of General Neurology, University Hospital Tübingen, Hoppe-Seyler-Strasse 3, 72076, Tübingen, Germany.
Michael
M
Weller
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D001932:Brain Neoplasms / Q000188:drug therapy* / Q000473:pathology; D003606:Dacarbazine / Q000008:administration & dosage / Q000031:analogs & derivatives*; D018572:Disease-Free Survival; D004806:Ependymoma / Q000188:drug therapy* / Q000473:pathology; D015251:Epirubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D053208:Kaplan-Meier Estimate; D008130:Lomustine / Q000008:administration & dosage; D008297:Male; D008875:Middle Aged; D011344:Procarbazine / Q000008:administration & dosage; D000077204:Temozolomide; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
D011344:Procarbazine; D015251:Epirubicin; D014750:Vincristine; D008130:Lomustine; D003606:Dacarbazine; D007069:Ifosfamide; D000077204:Temozolomide
Adults; Chemotherapy; Intracranial ependymoma; Overall survival; Progression-free survival