AI Article Synopsis
- Diffuse leptomeningeal glioneuronal tumors (DLGNTs) are rare childhood CNS tumors that cause symptoms like increased intracranial pressure and spinal cord compression, often showing widespread leptomeningeal enhancement on imaging.
- Microscopic analysis reveals oligodendroglial-like cells, and DLGNTs are characterized by specific genetic alterations, particularly involving the BRAF-KIAA1549 fusion.
- Treatment typically includes chemotherapy and radiation, but there is no standard grading system since the tumors can behave either indolently or aggressively.
Article Abstract
Diffuse leptomeningeal glioneuronal tumors (DLGNTs) are rare central nervous system tumors of childhood that were recently described as a new entity. DLGNTs usually manifest with symptoms related to increased intracranial pressure or spinal cord compression. The classic radiological feature is a widespread leptomeningeal enhancement that may involve the entire neuroaxis. Microscopic examination demonstrates oligodendroglial-like cells that are positive for OLIG2, MAP2, and S100 and negative for IDH-1. Anaplastic features occur in some cases. Molecularly, DLGNTs are characterized by chromosome arm 1p deletion and alteration of a mitogen-activated protein kinase (MAPK) pathway gene, most commonly BRAF-KIAA1549 fusion. There is no established grading system for these tumors, which may have an indolent or aggressive behavior. Treatment usually involves chemotherapy and radiation therapy.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1007/s00381-022-05625-1 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
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!