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BRAF inhibitor monotherapy in BRAFV600E-mutated pediatric low-grade glioma: a single center's experience.

Front Oncol

January 2025

Department of Pediatrics, Children's Healthcare of Atlanta, Atlanta, GA, United States.

Background: Pediatric low-grade gliomas (pLGGs) have an overall survival of over 90%; however, patients harboring a BRAF alteration may have worse outcomes, particularly when treated with classic chemotherapy. Combined BRAF/MEK inhibition following incomplete resection demonstrated improved outcome in BRAF altered pLGG compared to combined carboplatin/vincristine chemotherapy and is now considered the standard FDA-approved treatment for this group of tumors. The aim herein was to investigate the efficacy and tolerability of single agent BRAF inhibitor treatment in BRAF altered pLGG.

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Neuropathology of focal epilepsy: the promise of artificial intelligence and digital Neuropathology 3.0.

Pathology

December 2024

Partner of the European Reference Network (ERN) EpiCARE, Germany.

Focal lesions of the human neocortex often cause drug-resistant epilepsy, yet ​surgical resection of the epileptogenic region has been proven as a successful strategy to control seizures in a carefully selected patient cohort. Continuous efforts to study neurosurgically resected brain samples at the microscopic level, i.e.

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Low-grade gliomas and reactive piloid gliosis can present with overlapping features on conventional histology. Given the large implications for patient treatment, there is a need for effective methods to discriminate these morphologically similar but clinically distinct entities. Using routinely available stains, we hypothesize that a limited panel including SOX10, p16, and cyclin D1 may be useful in differentiating mitogen-activated protein (MAP) kinase-activated low-grade gliomas from piloid gliosis.

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Gangliogliomas (GG) are rare primary central nervous system (CNS) tumors. These CNS tumors are more commonly located at the supratentorial level. The treatment of choice for these tumors is surgical resection, and the role of radiotherapy remains controversial.

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Article Synopsis
  • - The article discusses a rare finding of atypical pathological features in the brains of patients with drug-resistant epilepsy, revealing a mix of ganglioglioma and focal cortical dysplasia, which hasn't been documented before.
  • - Comprehensive tests including histopathological staining and molecular genetic analysis were conducted, and MRI scans showed unusual patterns such as "transmantle" distribution and local alterations in the brain structure.
  • - The findings suggest the presence of a new subgroup of gangliogliomas characterized by cellular atypia, indicating a potential link between different types of neuronal-glial tumors that could reshape our understanding of epilepsy-related brain pathology.
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