Angiocentric glioma: A case report and review of the literature.

J Clin Neurosci

Department of Neurosurgery, Tongji Hospital, School of Medicine, Tongji University, Shanghai 200092, China. Electronic address:

Published: December 2021

Background: Angiocentric glioma (AG) is a rare, low-grade glioma with slow growth. In 2007, AG was first classified as a solid tumor according to the WHO classification of the central nervous system (WHO class I). The outcome and prognosis of most of the cases are very good, but a few cases with tumor metastasis and disease progression, even death, have been reported. We report a case and systematically analyze previous literature to increase our understanding of the disease and determine the factors that may affect disease progression to make prognostic judgments.

Case Presentation: A young male patient complained of a 3-year history of epilepsy. Anti-epileptic drug treatment was ineffective. An imaging examination revealed a lesion in the left parietal cortex area. Thus, the lesion was completely resected. The pathological diagnosis was angiocentric glioma. During a follow-up of two years, the patient had epilepsy relief controlled by sodium valproate and a disease-free period.

Conclusion: AG is an epilepsy-related low-grade glioma that heals after complete resection in most reported cases. However, few reported cases have had disease progression and death. This result may be due to the pathological complexity of the diseased tissue. In addition, AG is usually found to have an MYB-QKI rearrangement on genetic analysis. Due to the small number of reported cases and studies, our understanding and knowledge of this disease are still lacking. The potential malignant changes and prognostic factors need to be verified in more than clinical cases and basic research in the future.

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jocn.2021.10.016DOI Listing

Publication Analysis

Top Keywords

angiocentric glioma
12
disease progression
12
reported cases
12
low-grade glioma
8
progression death
8
cases
6
disease
5
glioma case
4
case report
4
report review
4

Similar Publications

Angiocentric glioma (AG) is a supratentorial diffuse low-grade glioma characterized by the MYB::QKI fusion gene, showing angiocentric growth of monomorphous spindle cells with astrocytic and ependymal immunophenotypes. We describe a rare case of MYB::QKI fusion-positive diffuse cerebellar glioma in a 54-year-old male. The patient initially presented with a T2/FLAIR hyperintense lesion in the left cerebellar hemisphere and slowly progressive neurological symptoms.

View Article and Find Full Text PDF

Diffuse pediatric-type high-grade gliomas (pedHGG), H3- and IDH-wildtype, encompass three main DNA-methylation-based subtypes: pedHGG-MYCN, pedHGG-RTK1A/B/C, and pedHGG-RTK2A/B. Since their first description in 2017 tumors of pedHGG-RTK2A/B have not been comprehensively characterized and clinical correlates remain elusive. In a recent series of pedHGG with a Gliomatosis cerebri (GC) growth pattern, an increased incidence of pedHGG-RTK2A/B (n = 18) was observed.

View Article and Find Full Text PDF

The World Health Organization (WHO) Central Nervous System (CNS) Tumors Classification 5 edition (2021) integrates both molecular and histopathological criteria for diagnosing glial tumors. This updated classification highlights significant differences between pediatric and adult gliomas in terms of molecular characteristics and prognostic implications. The 5 edition comprises a new category of pediatric-type diffuse low-grade glioma (PDLGG) and pediatric-type diffuse high-grade glioma (PDHGG), classified mainly based on genetic alterations and histopathological features.

View Article and Find Full Text PDF

MYB/MYBL1-altered gliomas frequently harbor truncations and non-productive fusions in the MYB and MYBL1 genes.

Acta Neuropathol

October 2024

Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 10 Center Dr., Room 2S235, Bethesda, MD, 20892, USA.

Astrocytomas that harbor recurrent genomic alterations in MYB or MYBL1 are a group of Pediatric-type diffuse low-grade gliomas that were newly recognized in the 2021 WHO Classification of Tumors of the Central Nervous System. These tumors are described in the WHO classification as harboring fusions in MYB or MYBL1. In this report, we examine 14 consecutive cases in which a MYB or MYBL1 alteration was identified, each with diagnostic confirmation by genome-wide DNA methylation profiling (6 Angiocentric gliomas and 8 Diffuse astrocytomas, MYB- or MYBL1-altered), for their specific genomic alterations in these genes.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!