Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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October 31, 2024
Diffuse Pediatric-Type High-Grade Glioma H3-Wild-Type and IDH-Wild-Type, Optico-Chiasmatic Region
- Background:
- Pediatric high-grade gliomas are relatively uncommon tumors and are considered a separate entity in the new WHO Classification of CNS Tumors. Although they are qualified as “diffuse,” a circumscribed pattern of growth may be encountered. Regardless of morphology, they are considered biologically aggressive tumors with a dismal prognosis. The presence of extensive meningeal spread of the tumor at presentation is a poor prognostic indicator.
- Clinical Presentation:
- Clinical features may vary depending on the location of the tumor.
- Key Diagnostic Features:
- Clinical suspicion of high-grade tumor is based on the short duration of symptoms and lack of constitutional symptoms to suggest infective etiology.
- Imaging features are not very specific.
- Inhomogeneously enhancing masses, often with rim enhancement have been described. Lesions may be well-demarcated and may have mild perilesional edema.
- Diffusion restriction is not a consistent feature.
- Differential Diagnosis:
- Tuberculosis: Constitutional symptoms often present, T2 hypointense lesions with peripheral enhancement with associated basal exudates and leptomeningeal enhancement
- Lymphoma: T2 iso-/hypointense lesions along the ventricular margins with diffusion restriction and homo-/heterogeneous enhancement
- Germ cell tumors: Lesions of T2 heterogeneous signal with foci of blooming and patchy diffusion restriction. Elevated serum levels of tumor markers like human chorionic gonadotrophin and alpha-fetoprotein may be present.
- Management:
- Tumor resection followed by radiotherapy and chemotherapy
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