title: Glioblastoma with PNET-like components has a higher frequency of isocitrate dehydrogenase 1 (IDH1) mutation and likely a better prognosis than primary glioblastoma.
abstract: Glioblastoma with primitive neuroectodermal tumor-like components (GBM-PNET), a rare variant of glioblastoma, poses both diagnostic and therapeutic challenges. Ten patients with GBM-PNET were investigated with a median age of 51.5 years and the 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 8 cases harbored isocitrate dehydrogenase 1 (IDH1) R132H mutation, while IDH2 R172 mutations were not identified. Three out of 10 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.
authors.affiliation: Department of Pathology, Hartford Hospital, CT, USA.
authors.forename: Xianyuan
authors.initials: X
authors.lastname: Song
authors.forename: R
authors.initials: R
authors.lastname: Andrew Allen
authors.forename: S
authors.initials: S
authors.lastname: Terence Dunn
authors.forename: Kar-Ming
authors.initials: KM
authors.lastname: Fung
authors.forename: Peter
authors.initials: P
authors.lastname: Farmer
authors.forename: Shital
authors.initials: S
authors.lastname: Gandhi
authors.forename: Tulika
authors.initials: T
authors.lastname: Ranjan
authors.forename: Alexis
authors.initials: A
authors.lastname: Demopoulos
authors.forename: Marc
authors.initials: M
authors.lastname: Symons
authors.forename: Michael
authors.initials: M
authors.lastname: Schulder
authors.forename: Jian Yi
authors.initials: JY
authors.lastname: Li
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001932:Brain Neoplasms / Q000737:chemistry / Q000201:enzymology / Q000235:genetics* / Q000473:pathology; D019002:CD56 Antigen / Q000032:analysis; D004252:DNA Mutational Analysis; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D005909:Glioblastoma / Q000737:chemistry / Q000201:enzymology / Q000235:genetics* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007521:Isocitrate Dehydrogenase / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D018193:Neoplasms, Complex and Mixed / Q000737:chemistry / Q000201:enzymology / Q000235:genetics* / Q000473:pathology; D018242:Neuroectodermal Tumors, Primitive / Q000737:chemistry / Q000201:enzymology / Q000235:genetics* / Q000473:pathology; D011379:Prognosis; D016019:Survival Analysis; D015996:Survival Rate; D013997:Time Factors; D016159:Tumor Suppressor Protein p53 / Q000032:analysis; D014746:Vimentin / Q000032:analysis
chemical_list: D019002:CD56 Antigen; C522918:NCAM1 protein, human; C495901:TP53 protein, human; D016159:Tumor Suppressor Protein p53; D014746:Vimentin; D007521:Isocitrate Dehydrogenase; C543588:IDH1 protein, human
references.citation: Brain Tumor Pathol. 2002;19(2):51-8
references.pmid: 12622133
references.citation: Acta Neuropathol. 2002 Feb;103(2):193-8
references.pmid: 11810187
references.citation: Arch Pathol Lab Med. 1997 May;121(5):485-92
references.pmid: 9167602
references.citation: J Mol Diagn. 2010 Nov;12(6):750-6
references.pmid: 20847279
references.citation: Acta Neuropathol. 2008 Dec;116(6):597-602
references.pmid: 18985363
references.citation: Cancer Cell. 2007 Jan;11(1):69-82
references.pmid: 17222791
references.citation: Clin Neuropathol. 2004 Sep-Oct;23(5):218-22
references.pmid: 15581024
references.citation: Am J Surg Pathol. 2011 Apr;35(4):573-82
references.pmid: 21378543
references.citation: J Pediatr Hematol Oncol. 2006 Nov;28(11):703-10
references.pmid: 17114955
references.citation: Virchows Arch. 2005 Sep;447(3):665-8
references.pmid: 15968544
references.citation: Acta Neurol Scand. 2010 Sep;122(3):159-67
references.pmid: 20298491
references.citation: Neuroradiology. 2001 Nov;43(11):927-33
references.pmid: 11760795
references.citation: Acta Neuropathol. 2010 Apr;119(4):501-7
references.pmid: 20131059
references.citation: Pathol Int. 1995 Apr;45(4):275-82
references.pmid: 7550996
references.citation: Am J Pathol. 2007 May;170(5):1445-53
references.pmid: 17456751
references.citation: Brain Pathol. 2009 Jan;19(1):81-90
references.pmid: 18452568
references.citation: J Neurooncol. 2007 Jul;83(3):313-8
references.pmid: 17406789
references.citation: Cell Cycle. 2009 Jun 1;8(11):1806-7
references.pmid: 19411854
references.citation: Clin Neuropathol. 2001 Sep-Oct;20(5):212-8
references.pmid: 11594506
references.citation: Diagn Cytopathol. 2009 Dec;37(12):906-9
references.pmid: 19582814
references.citation: Clin Neuropathol. 2002 Jul-Aug;21(4):193-5; author reply 195-6
references.pmid: 12143929
references.citation: Nat Rev Cancer. 2007 Oct;7(10):733-6
references.pmid: 17882276
references.citation: Oncogene. 2010 Dec 9;29(49):6409-17
references.pmid: 20972461
references.citation: Cell Mol Neurobiol. 2009 Sep;29(6-7):845-58
references.pmid: 19288188
references.citation: N Engl J Med. 2009 Feb 19;360(8):765-73
references.pmid: 19228619
references.citation: Pathol Int. 2008 Jun;58(6):396-401
references.pmid: 18477220
references.citation: Neurol Med Chir (Tokyo). 2005 Oct;45(10):530-5
references.pmid: 16247240
references.citation: N Engl J Med. 2005 Mar 10;352(10):987-96
references.pmid: 15758009
references.citation: J Clin Neurosci. 2005 May;12(4):492-5
references.pmid: 15925794
references.citation: J Neurooncol. 2008 Jan;86(2):217-24
references.pmid: 17713720