Low-grade epilepsy-associated tumors (LEATs) are a distinct group of tumors commonly encountered in pediatric drug-resistant epilepsy that necessitate surgical intervention. Like tumors elsewhere in the central nervous system, molecular characterization is becoming an increasingly important consideration in pediatric neuro-oncology prognostication and management for LEATs. Thus, familiarity with relevant tumor mutations and radiogenomic features of LEATs is important for radiologists caring for affected patients. This article will review the genetic alterations and imaging characteristics of LEATs, formatted according to the three categories defined by the World Health Organization (WHO): glioneuronal and neuronal tumors (ganglioglioma, dysembryoplastic neuroepithelial tumor, papillary glioneuronal tumor, multinodular and vacuolating neuronal tumor); circumscribed astrocytic gliomas (pilocytic astrocytoma, pleomorphic xanthoastrocytoma); and pediatric-type diffuse low-grade gliomas (diffuse astrocytoma MYB or MYBL1-altered, angiocentric glioma, diffuse low-grade glioma MAPK pathway-altered, polymorphous low-grade neuroepithelial tumor of the young).

Pediatric diffuse low-grade gliomas (LGGs) are heterogeneous group of central nervous system tumors that typically exhibit a relatively benign clinical course. These tumors represent a unique classification in pediatric neuro-oncology, distinct from adult counterparts in terms of biological behavior, molecular pathways, and clinical presentation. The evolving World Health Organization classification system has increasingly relied on combination of histopathologic, genetic, and radiologic criteria to define and distinguish among pediatric glioma subtypes. This article aims to synthesize current knowledge regarding the clinical, molecular, and radiologic features of these pediatric diffuse LGGs and highlights the nuances in diagnosis, treatment approaches, and prognostic outlook.

Angiocentric glioma (AG), a benign tumor identified within the last two decades, was officially included in the 2007 WHO Classification of Tumors of the Central Nervous System, WHO grade I. The tumor is relatively rare, with only approximately 100 cases reported. We aim to complement the characteristics and long-term prognosis of AG, as well as to detect MYB-QKI fusions. The characteristics of all cases collected between 1 March 2009 and 1 March 2023 at the Beijing Sanbo Brain Hospital, Capital Medical University, were summarized and analyzed. Additionally, all fourteen patients were tested for MYB-QKI fusions. AG more predominantly occurs in adolescents (median age 16.5-year-old), and commonly presents with drug-resistant epilepsy. AG is frequently localized in the supratentorial regions and only one patient is in the brainstem. Brain parenchyma atrophy, and stalk-like signs can observe in imaging. Pathologically, tumor cells are perivascular pseudorosettes, presenting immunoreactivity for GFAP, S-100, Vimentin, "dot-like" staining for EMA, and low proliferative activity. Focal cortex dysplasia was observed in four patients. Twelve of fourteen (85.7%) patients were found with MYB-QKI fusions. Completely surgical resection typically has a satisfactory prognosis with long-term follow-up. AG is a rare benign tumor with a favorable prognosis after complete resection, characterized by refractory epilepsy, frequently occurring in adolescents. MYB-QKI fusions were detected in most AG patients, as a good defining genetic alteration pathologically. The potential presence of focal cortical dysplasia (FCD) may affect the prognosis of epilepsy.

Angiocentric glioma (AG) is a very rare neoplasm. Limited literature described this rare lesion and most of them are case reports. Here we report 21 patients with AG and review the literature. Clinical data from the 21 patients who underwent surgical treatment in our institute between 2015 and 2025 were reviewed. We also searched PubMed database between 2005 and 2025 using the keywords "angiocentric glioma" or "angiocentric gliomas" and 74 patients were reviewed. The authors' cohort include 12 males and 9 females, with a mean age of 17.9 ± 17.9 years. Gross-total resection (GTR) and non-GTR were achieved in 15 and 6 patients, respectively. After a mean follow-up of 41.7 ± 31.4 months, no patient died and tumor recurrence occurred in 1 patient. In the literature between 2005 and 2025, 74 cases of AG were identified. Among them, 46 cases were males and 28 cases were females with a mean age of 14.7 ± 13.9 years. GTR, non-GTR and biopsy were achieved in 49, 17, 7 cases, respectively. After a mean follow-up of 29.4 ± 31.6 months, 2 patients died, for a mean follow-up of 32.7 ± 36.1 months, 5 patients suffered tumor recurrence. Among all cases that underwent immunohistochemical examination, 98.5% were positive for Glial Fibrillary Acidic Protein (GFAP) and 82.1% were positive for Epithelial Membrane Antigen (EMA). Genetic testing was performed in a total of 6 cases and all of them showed MYB-QKI gene fusion. Progression-Free Survival (PFS) rates at 1, 5, 10 years were 96%, 94% and 77% respectively, and Overall Survival (OS) rates at 1, 5, 10 years were 99%, 99% and 91%. AGs are rare tumors with a higher tendency in males and they usually involved the frontal and temporal lobe. In immunohistochemical studies, GFAP and EMA are frequently positive. The MYB-QKI gene fusion is a characteristic feature of this tumor. GTR was regarded as the best treatment. Although most AGs were clinically indolent, a few unusual cases showed aggressive behaviors. A larger cohort with long-term follow-up is necessary to verify our findings.

We performed a systematic review of the literature to better define the scope of MYB alterations in angiocentric glioma and their associated clinical characteristics, as well as to include a novel MYB mutation in an angiocentric glioma case. We also review MYB alterations in the context of oncologic disease. Following PRISMA guidelines, we searched PubMed and Web of Science for relevant literature from 2010 to October 2024. Included articles reported original data on human subjects with angiocentric glioma and a detected MYB mutation. We include one additional angiocentric glioma case showcasing a novel MYB mutation. A total of 14 studies met the inclusion criteria, with a total of 114 patients with individual data for pooled analysis. The mean age was 10.3 years (SD ±9.7 years); 60% of patients were male. MYB::QKI was the most common fusion in 68% of patients. Other MYB mutations included MYB rearrangements, MYB::ESR1, MYB::PCDHGA1, MYB::LOC105378099, and MYB::MMP16. The most common anatomical location was in the cerebral cortex in 68% of patients. MYB fusions in other relevant neuro-oncologic diseases highlight the importance of MYB fusions in adenoid cystic carcinomas, which frequently occur at the skull base, head and neck, and breast. In conclusion, we characterize the breadth of angiocentric glioma patterns in terms of demographics, anatomic location, and MYB fusion patterns. The updated molecular diagnosis of angiocentric glioma as of 2021 warrants continued exploration of the scope of MYB oncogene fusions as drivers of prognosis and targets for future therapies.