J Korean Neurosurg Soc.  2016 Jan;59(1):44-51. 10.3340/jkns.2016.59.1.44.

Malignant Glioma with Neuronal Marker Expression : A Clinicopathological Study of 18 Cases

• Kim HR
• Lee JJ
• Lee JI
• Nam DH
• Suh YL
• Seol HJ

• KMID: 2152917
• DOI: http://doi.org/10.3340/jkns.2016.59.1.44

Abstract

OBJECTIVE
Malignant gliomas with neuronal marker expression (MGwNM) are rare and poorly characterized. Increasingly diverse types of MGwNM have been described and these reported cases underscore the dilemmas in the classification and diagnosis of those tumors. The aim of this study is to provide additional insights into MGwNM and present the clinicopathological features of 18 patients.
METHODS
We reviewed the medical records of 18 patients diagnosed as MGwNM at our institute between January 2006 and December 2012. Macroscopic total resection was performed in 11 patients (61%). We evaluated the methylation status of O6-methylguanine-DNA methyltransferase (MGMT) and expression of isocitrate dehydrogenase 1 (IDH-1) in all cases, and deletions of 1p and 19q in available cases.
RESULTS
The estimated median overall survival was 21.2 months. The median progression-free survival was 6.3 months. Six patients (33%) had MGMT methylation but IDH1 mutation was found in only one patient (6%). Gene analysis for 1p19q performed in nine patients revealed no deletion in six, 19q deletion only in two, and 1p deletion only in one. The extent of resection was significantly correlated with progression free survival on both univariate analysis and multivariate analysis (p=0.002 and p=0.013, respectively).
CONCLUSION
In this study, the overall survival of MGwNM was not superior to glioblastoma. The extent of resection has a significant prognostic impact on progression-free survival. Further studies of the prognostic factors related to chemo-radio therapy, similar to studies with glioblastoma, are mandatory to improve survival.

Keyword

Malignant gliomas; Neuronal marker; Methylation status of O6-methylguanine-DNA methyltransferase; Expression of isocitrate dehydrogenase 1; 1p and 19q

MeSH Terms

Classification
Diagnosis
Disease-Free Survival
Glioblastoma
Glioma*
Humans
Isocitrate Dehydrogenase
Medical Records
Methylation
Multivariate Analysis
Neurons*
Isocitrate Dehydrogenase

Figure

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  Fig. 1 Histologic, immunohistochemical and gene analysis of malignant glioneuronal tumor. A : Hematoxylin-eosin slides of fifteen cases show high grade glial tumor with neuronal component. B : Positive immunohistochemical stains of GFAP, synaptophysin, neurofilament, NeuN, and IDH-1 (a : GFAP, b : synaptophysin, c : neurofilament, d : NeuN, e : IDH-1) and the result of immunohistochemical and gene analysis of 18 patients with malignant glioneuronal tumors. C : Loss of chromosomes 1p and 19q by fluorescence in situ hybridization by using dual color probes (Vysis®LSI®1p36/1q25 and 19q13/19p13). a : two green 1q25 signals and 1 orange 1p36 signal, b : two green 19p13 signals and 1 orange 19q13 signal. NeuN : neuronal nuclear antigen, IDH-1 : isocitrate dehydrogenase 1, GFAP : glial fibrillary acidic protein.

• 

  Fig. 2 Graphs show overall survival (OS) curve (A) and progression-free survival (PFS) curve (B) according to age, extent of resection (C and D), size of tumor (E and F). TR : total removal, PR : partial removal.Graphs show MGMT methylation status (G and H).

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