Malignant progression of SEGA-imitating fibrous meningioma in a child carrying a germline CHEK2 mutation | Synapse
March 3, 2026
Malignant progression of SEGA-imitating fibrous meningioma in a child carrying a germline CHEK2 mutation
Key Points
Malignant progression was linked to constitutional CHEK2 mutations and somatic NF1 defects, indicating a genetic predisposition.
The initial response to mTOR inhibitors was favorable, suggesting potential therapeutic pathways for similar tumors.
Assessment using genetic testing revealed both CHEK2 and NF1 mutations, critical for understanding tumor behavior.
The findings highlight the importance of genetic factors in the malignant transformation of brain tumors.
Abstract
Constitutional CHEK2 mutations combined with somatic NF1 defect may have promoted the malignant progression of SEGA-imitating fibrous meningioma and its favorable initial response to mTOR inhibitors.