IJCEP Copyright © 2007-All rights reserved.
Int J Clin Exp Pathol 2011;4(7):xxx-xxx

Original Article
Glioblastoma with PNET-like components has a higher frequency of isocitrate
dehydrogenase 1 (IDH1) mutation and likely a better prognosis than primary glioblastoma

Xianyuan Song, R. Andrew Allen, S. Terence Dunn, Kar-Ming Fung, Peter Farmer, Shital Gandhi, Tulika Ranjan, Alexis Demopoulos, Marc
Symons, Michael Schulder, Jian Yi Li

Department of Pathology, Hartford Hospital, Hartford, CT, USA; Department of Pathology, The University of Oklahoma Health Sciences Center,
Oklahoma City, OK, USA; Department of Pathology and Lab. Medicine, North Shore-Long Island Jewish Health System, Lake Success, Hofstra
North Shore-LIJ School of Medicine, NY, USA; Department of Radiology, Neurology, 8Neurosurgery, North Shore University Hospital,
Manhasset, NY, USA; Tisch Cancer Institute, Mount Sinai School of Medicine, NY, USA; Center for Oncology and Cell Biology, The Feinstein
Institute for Medical Research, Manhasset, NY, USA.

Received August 18, 2011; accepted September 6, 2011; Epub September 17, 2011; published October 31, 2011

Abstract: Glioblastoma with primitive neuroectodermal tumor-like components (GBM-PNET), a rare variant of glioblastoma, poses both a
diagnostic and therapeutic challenge.  Ten patients with GBM-PNET were investigated with a median age of 51.5 years and male to female
ratio of 4:1.  The majority of patients (7 out of 10) showed ring-enhancing lesions on magnetic resonance imaging (MRI), which is classic for
GBMs. Restricted diffusion was noted in 7 cases where diffusion weighted imaging (DWI) was performed, which correlates with the presence
of PNET-like components. CD56 and vimentin immunostaining made the diagnosis of GBM-PNET much easier. Vimentin strongly and diffusely
highlighted the astrocytic components and was negative in PNET-like components, while CD56 was strongly and diffusely positive in both
astrocytic and PNET-like components. Seven out of 9 cases were positive for p53 in both astrocytic and PNET-like components. Two out of
eight cases harbored isocitrate dehydrogenase 1 (IDH1) R132H mutation, while IDH2 R172 mutations were not identified. Three out of the ten
patients had a median survival time of 17 months while the two patients, whose tumor carried IDH1 mutation, were still alive after 15 and 31
months of follow-up. Compared to primary GBMs, GBM-PNETs might have a better prognosis. Further large scale studies are necessary to
confirm this observation. (IJCEP11080017).

Keywords: Glioblastoma with PNET-like components, isocitrate dehydrogenase 1 (IDH1), glioblastoma, vimentin, CD56, diffusion weighted
imaging (DWI)

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Address all correspondence to:
Jian Yi Li, MD, PhD
Department of Pathology and Lab. Medicine
North Shore-Long Island Jewish Health System
6 Ohio Drive, Suite 202, Lake Success, NY 11042, USA.
Tel: 516-304-7240
Fax: 516-224-8586