IJCEP Copyright © 2007-All rights reserved.
Int J Clin Exp Pathol 2012;5(5):458-462

Case Report
Giant cell ependymoma-report of three cases and review of the literature

Jian Yi Li, Jose I Lopez, Suzanne Z Powell, Stephen W Coons, Gregory N Fuller

Department of Pathology and Lab. Medicine, North Shore and Long Island Jewish Health System, Lake Success, Hofstra North Shore-LIJ
School of Medicine, NY, USA; Department of Pathology, Hospital Universitario de Cruces, The University of the Basque Country (UPV/EHU),
Barakaldo, Bizkaia, Spain; Department of Pathology, The Methodist Hospital, Houston, TX, USA; Division of Neuropathology, Barrow
Neurological Institute, Phoenix, AZ, USA; Department of Pathology, The Univ. of Texas, M. D. Anderson Cancer Center, Houston, TX, USA

Received March 11, 2012; accepted April 21, 2012; Epub May 5, 2012; Published June 30, 2012

Abstract: Ependymomas constitute the most common type of primary spinal cord tumor, and are subclassified as myxopapillary
ependymoma, classic ependymoma, and anaplastic ependymoma. Ependymomas can be further subclassified based on morphologic
phenotype: cellular, papillary, tanycytic, clear cell, pigmented and epithelioid. Giant cell ependymoma (GCE), a rare variant, has recently been
described. Reported cases have exhibited a wide anatomic distribution, including spinal cord, cerebrum, and cerebellum. We report here three
cases of GCE, arising from cerebrum in a 5-year-old girl, spinal cord in a 34-year-old female and cerebellum in an 86-year-old female
respectively. Histologically those cases showed prominent pleomorphic giant cells with focal perivascular pseudorosettes in all cases. Tumor
cells were immunopositive for GFAP and EMA. Only the first case was qualified for anaplastic ependymoma. No recurrence was noted in these
three cases after 57, 46 and 6 months of follow-up respectively. By reviewing the literature, GCEs arising from spinal cord and cerebellum
tended to have low-grade morphology while supratentorially located GCEs tended to have anaplastic features. GCEs were preferentially
located in extraventricular regions. Anaplastic GCEs in adult population seemed to pursue a more aggressive behavior. Gross total resection
should still be the main treatment for GCEs. (IJCEP1203006)

Keywords: Ependymoma, giant cell ependymoma, perivascular pseudorosette, pleomorphic giant cell

Address all correspondence to:
Dr. Jian Yi Li
Department of Pathology and Laboratory Medicine
North Shore-Long Island Jewish Health System
6 Ohio Drive, Suite 202, Lake Success, NY 11042, USA.
Tel: +1 516-304-7240; Fax: +1 516-224-8586
E-mail: jli2@nshs.edu