Ependymoblastoma

from Wikipedia, the free encyclopedia
Histopathology of the ependymoblastoma.
Numerous so-called ependymoblastomatous rosettes are characteristic. [Hematoxylin-eosin stain]
Ependymoblastomatous rosettes. At a higher magnification, the densely packed, multi-row tumor cells around a central lumen are clearly visible. Hematoxylin-eosin stain.

As Ependymoblastom a very rare malignant is Brain Tumor from the group of embryonic tumors called, occurs particularly in newborns and infants. Like other more common embryonic tumors, ependymoblastoma is classified as grade IV according to the WHO classification of tumors of the central nervous system ; However, given the rarity of the tumor, reliable statements on the prognosis are not possible.

Neuropathology

Neuropathologically , the development of multi-row rosettes , which are referred to as ependymoblastomatous rosettes, is characteristic of the tumor . In view of the observation that ependymoblastomatous rosettes can also occur in other embryonic tumors, the importance of the ependymoblastoma as an independent entity was controversial.

Molecular genetics

The detection of an amplification on the long arm of chromosome 19 , gene locus q13.42, is characteristic and moves the ependymoblastoma into the vicinity of the so-called embryonic tumor with abundant neuropil and true rosettes .

literature

  • McLendon et al. a .: Central nervous system primitive neuroectodermal tumors. in: Louis u. a. (Editor) WHO Classification of Tumors of the Central Nervous System. Lyon, IARC Press 2007, ISBN 9283224302

Individual evidence

  1. ^ AR Judkins, DW Ellison: Ependymoblastoma: dear, damned, distracting diagnosis, farewell! *. In: Brain Pathology . 20, 2010, pp. 133-139, PMID 19120373 .
  2. ↑ A. Korshunov, M. Remke, M. Gessi, M. Ryzhova, T. Hielscher, H. Witt, V. Tobias, AM Buccoliero, I. Sardi, MP Gardiman, J. Bonnin, B. Scheithauer, AE Kulozik, O. Witt, S. Mork, A. von Deimling, OD Wiestler, F. Giangaspero, M. Rosenblum, T. Pietsch, P. Lichter, SM Pfister: Focal genomic amplification at 19q13.42 comprises a powerful diagnostic marker for embryonal tumors with ependymoblastic rosettes. In: Acta Neuropathologica . 120, 2010, pp. 253-260, PMID 20407781 .