[H3/IDH wildtype paediatric-type high-grade glioma(RTK1): a clinicopathological study]

Objective: To explore the pathogenesis, clinicopathological and molecular genetic features of H3/IDH wildtype, paediatric-type high-grade glioma (RTK1). Methods: A total of five cases diagnosed by the clinical features,imaging,histopathology,molecular genetics and prognosis from the Department of Pathology, the First Medical Center of PLA General Hospital were collected(2022-2025). Results: Among the five cases, three were female and two were male, aged 5-38 years,the median age is 8 years old.Tumors were located in the left/right frontal lobe, cerebellum brainstem, and right temporal lobe, respectively. The poor limb movement, unstable walking, headache accompanied by nausea, vomiting in five cases. Histopathology shows features of high-grade gliomas histological changes characterized by densely arranged cells with cell atypia, vascular proliferation,necrosis and mitotic activity. Molecular showed of PDGFRA amplification or mutation in five cases, accompanied by MGMT methylation, TERT, TP53 mutation. The total course of disease from onset to death in one case is about 10 years, indicating that the progression of the disease is slower than that of adult high-grade gliomas. Conclusions: Pediatric-type gliomas occur predominantly in children but can also be observed in adults. Their disease progression and prognosis are generally more favorable compared to adult-type high-grade gliomas. Molecular testing plays a crucial role in diagnosis and differential diagnosis, holding significant importance for treatment and prognosis evaluation.