Diffuse Leptomeningeal Glioneuronal Tumor in a 4.5-year-old Girl: A Case Report and Review of the Literature

2020 ◽  
Author(s):  
Anna Gabryś ◽  
Julia Kuzaj ◽  
Dominika Pawełczak ◽  
Katarzyna Seliga ◽  
Agnieszka Jelińska ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
World Health ◽  
Glioneuronal Tumor ◽  
Molecular Testing ◽  
Diffuse Leptomeningeal Glioneuronal Tumor ◽  
History Of ◽  
The Central Nervous System ◽  
Classification Of Tumors ◽  
Health Organization

AbstractDiffuse leptomeningeal glioneuronal tumor (DLGNT) is an entity introduced in 2016 World Health Organization classification of tumors of the central nervous system. The tumor occurs very rarely. Due to the lack of specific clinical and radiological features, biopsy is necessary to be performed and histological and immunohistochemical testing is essential to reach the diagnosis. A 4.5-year-old girl presented with a history of headache, vomiting, and right eye convergent squint. Imaging revealed multiple enhancing lesions located supra- and infratentorially and intramedullary. Histopathological examination demonstrated diffused growth of neoplastic cells. Molecular testing revealed KIAA1549-BRAF fusion and the diagnosis of DLGNT was stated.

scholarly journals The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary

2016 ◽  
Vol 131 (6) ◽  
pp. 803-820 ◽  
Author(s):  
David N. Louis ◽  
Arie Perry ◽  
Guido Reifenberger ◽  
Andreas von Deimling ◽  
Dominique Figarella-Branger ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
World Health Organization ◽  
World Health ◽  
World Health Organization Classification ◽  
System A ◽  
The Central Nervous System ◽  
Classification Of Tumors ◽  
Health Organization

scholarly journals What is New in 2019 World Health Organization (WHO) Classification of Tumors of the Digestive System: Review of Selected Updates on Neuroendocrine Neoplasms, Appendiceal Tumors, and Molecular Testing

2020 ◽  
Author(s):  
Naziheh Assarzadegan ◽  
Elizabeth Montgomery
Keyword(s):  
World Health Organization ◽  
Digestive System ◽  
English Literature ◽  
World Health ◽  
Response To Treatment ◽  
Neuroendocrine Neoplasms ◽  
Molecular Testing ◽  
Classification Of Tumors ◽  
Health Organization

Context.— The 5th edition of the World Health Organization classification of digestive system tumors discusses several advancements and developments in understanding the etiology, pathogenesis, and diagnosis of several digestive tract tumors. Objective.— To provide a summary of the updates with a focus on neuroendocrine neoplasms, appendiceal tumors, and the molecular advances in tumors of the digestive system. Data Sources.— English literature and personal experiences. Conclusions.— Some of the particularly important updates in the 5th edition are the alterations made in the classification of neuroendocrine neoplasms, understanding of pathogenesis of appendiceal tumors and their precursor lesions, and the expanded role of molecular pathology in establishing an accurate diagnosis or predicting prognosis and response to treatment.

scholarly journals EGFRAmplification andIDHMutations in Glioblastoma Patients of the Northeast of Morocco

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Nadia Senhaji ◽  
Sara Louati ◽  
Laila Chbani ◽  
Hind El Fatemi ◽  
Nawal Hammas ◽  
...  
Keyword(s):  
University Hospital ◽  
World Health ◽  
World Health Organization Classification ◽  
Histological Criteria ◽  
The Central Nervous System ◽  
Classification Of Tumors ◽  
Health Organization ◽  
Exon 20 ◽  
The University

Glioblastomas are the most frequent and aggressive primary brain tumors which are expressing various evolutions, aggressiveness, and prognosis. Thus, the 2007 World Health Organization classification based solely on the histological criteria is no longer sufficient. It should be complemented by molecular analysis for a true histomolecular classification. The new 2016 WHO classification of tumors of the central nervous system uses molecular parameters in addition to histology to reclassify these tumors and reduce the interobserver variability. The aim of this study is to determine the prevalence ofIDHmutations andEGFRamplifications in the population of the northeast region of Morocco and then to compare the results with other studies.Methods.IDH1codon 132 andIDH2codon 172 were directly sequenced and the amplification of exon 20 ofEGFRgene was investigated by qPCR in 65 glioblastoma tumors diagnosed at the University Hospital of Fez between 2010 and 2014.Results. The R132HIDH1mutation was observed in 8 of 65 tumor samples (12.31%). No mutation ofIDH2was detected.EGFRamplification was identified in 17 cases (26.15%).Conclusion. A systematic search of both histological and molecular markers should be requisite for a good diagnosis and a better management of glioblastomas.

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