Document Detail

Glioblastomas with giant cell and sarcomatous features in patients with turcot syndrome type 1: a clinicopathological study of 3 cases.
MedLine Citation:
PMID:  20657316     Owner:  NLM     Status:  In-Process    
BACKGROUND: Turcot syndrome (TS) is a rare genetic disorder of DNA mismatch repair predisposing to glioblastoma (GBM) in the type 1 variant.
OBJECTIVE: We report the clinicopathological and genetic features of 3 gliomas in TS type 1 patients.
METHODS: Three cases were reviewed from our clinical and pathology files at Washington University with the diagnosis of TS 1 and GBM over the past 14 years. All 3 had classic features of GBM, but also demonstrated bizarre multinucleated giant cells and remarkably high mitotic indices. Sarcomatous regions were found in 2. Despite these features, the patients had prolonged survival times of 44, 55, and >29 months (ie, currently alive). Demographic and clinical courses were abstracted from retrospective chart review. Histopathology was reviewed from all cases and reticulin histochemistry was added to identify possible foci of sarcomatous differentiation.
RESULTS: All 3 had classic features of GBM, and Ki-67 labeling indices ranged from 18 to 45%. All 3 also showed strong nuclear p53 positivity. Two cases were negative for the isocitrate dehydrogenase 1 (IDH1) mutation, and O-Methylguanine methyltransferase promoter methylation was seen in one. Fluorescence in situ hybridization was done using 1p/1q, 19p/19q, centromere 7/epithelial growth factor receptor (EGFR), and PTEN/DMBT1 probes. Focal EGFR amplification was seen in one case, although other common alterations of either primary GBMs or gliomas with prolonged survival (1p/19q codeletion) were lacking.
CONCLUSION: We conclude that 1) the giant cell variant of GBM is overrepresented in TS; 2) gliosarcomas may also be encountered; and 3) survival is often favorable, despite histological anaplasia and exuberant proliferation.
Eriks A Lusis; Sarah Travers; Sarah C Jost; Arie Perry
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Neurosurgery     Volume:  67     ISSN:  1524-4040     ISO Abbreviation:  Neurosurgery     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2010-08-23     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7802914     Medline TA:  Neurosurgery     Country:  United States    
Other Details:
Languages:  eng     Pagination:  811-7; discussion 817     Citation Subset:  IM    
Department of Neurosurgery, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
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