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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August 7, 2014
Desmoplastic Infantile Ganglioglioma
- Rare, usually benign, supratentorial tumor with a favorable prognosis.
- Most common clinical presentation is a rapidly increasing head circumference.
- Usually arise in children less than 18 months of age, and boys are more commonly affected.
- Key Diagnostic Features:
- Characterized by a radiologically malignant appearance.
- Superficial location is common.
- Frontal and parietal lobes are often involved.
- Massive, cystic supratentorial lesion with avidly enhancing solid component along the cortical margin of the mass, with a firm dural attachment
- Leptomeningeal enhancement and thickening may aid in the diagnosis.
- DDx:
- Pleomorphic xanthoastrocytoma
- Primitive neuroectodermal tumor
- Supratentorial ependymoma
- Typical ganglioglioma
- Hemangioblastoma
- Pilocytic astrocytoma
- Dysembryoplastic neuroepithelial tumor
- Rx: Total surgical resection is usually curative with no further additional therapy.
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