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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December 21, 2015
Glioblastoma Multiforme of the Pineal Region
- Background:
- Pineal region tumors most frequently comprise three categories: germ cell, parenchymal cell, and glial tumors.
- They make up less than 1% of intracranial tumors in adults.
- Most pineal gliomas are low-grade astrocytomas.
- Glioblastoma multiforme (GBM) tumors are exceedingly rare tumors in the pineal region, with approximately 65 cases reported.
- The clinical and radiologic characteristics of pineal GBM does not differentiate it from other malignancies of this region; thus, surgical biopsy is generally required for definitive diagnosis.
- Clinical Presentation:
- Symptoms are related to increased intracranial pressure/hydrocephalus and ophthalmologic symptoms (especially Parinaud syndrome).
- Key Diagnostic Features:
- Heterogeneously enhancing mass with a necrotic component involving the pineal region, and infiltration into the surrounding structures, such as the midbrain and thalamus.
- Pineal region GBMs do not show a higher rate of leptomeningeal or ependymal dissemination compared to other high-grade pineal tumors.
- Differential Diagnosis:
- There are no imaging findings that can make the diagnosis prospectively, although ill defined infiltrative non-enhancing involvement of the brain parenchyma (thalami, insula) may raise the suspicion of a GBM or an infiltrative astrocytoma.
- Germ cell tumors (most common)
- Pineal parenchymal tumors
- Astrocytoma of the pineal gland
- Pineal metastasis
- Treatment:
- Surgical resection followed by radiation therapy and chemotherapy
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