TY  - JOUR
AU  - Rudà, Roberta
AU  - Reifenberger, Guido
AU  - Frappaz, Didier
AU  - Pfister, Stefan
AU  - Laprie, Anne
AU  - Santarius, Thomas
AU  - Roth, Patrick
AU  - Tonn, Joerg Christian
AU  - Soffietti, Riccardo
AU  - Weller, Michael
AU  - Moyal, Elizabeth Cohen-Jonathan
TI  - EANO guidelines for the diagnosis and treatment of ependymal tumors.
JO  - Neuro-Oncology
VL  - 20
IS  - 4
SN  - 1523-5866
CY  - Oxford
PB  - Oxford Univ. Press
M1  - DKFZ-2018-00498
SP  - 445 - 456
PY  - 2018
AB  - Ependymal tumors are rare CNS tumors and may occur at any age, but their proportion among primary brain tumors is highest in children and young adults. Thus, the level of evidence of diagnostic and therapeutic interventions is higher in the pediatric compared with the adult patient population.The diagnosis and disease staging is performed by craniospinal MRI. Tumor classification is achieved by histological and molecular diagnostic assessment of tissue specimens according to the World Health Organization (WHO) classification 2016. Surgery is the crucial initial treatment in both children and adults. In pediatric patients with intracranial ependymomas of WHO grades II or III, surgery is followed by local radiotherapy regardless of residual tumor volume. In adults, radiotherapy is employed in patients with anaplastic ependymoma WHO grade III, and in case of incomplete resection of WHO grade II ependymoma. Chemotherapy alone is reserved for young children <12 months and for adults with recurrent disease when further surgery and irradiation are no longer feasible. A gross total resection is the mainstay of treatment in spinal ependymomas, and radiotherapy is reserved for incompletely resected tumors. Nine subgroups of ependymal tumors across different anatomical compartments (supratentorial, posterior fossa, spinal) and patient ages have been identified with distinct genetic and epigenetic alterations, and with distinct outcomes. These findings may lead to more precise diagnostic and prognostic assessments, molecular subgroup-adapted therapies, and eventually new recommendations pending validation in prospective studies.
LB  - PUB:(DE-HGF)16
C6  - pmid:29194500
C2  - pmc:PMC5909649
DO  - DOI:10.1093/neuonc/nox166
UR  - https://inrepo02.dkfz.de/record/132855
ER  -