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Virtual Pediatric Hospital: Paediapaedia: Craniopharyngioma Paediapaedia: Neurological Diseases


Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Clinical Presentation:
May have headaches and increased intracranial pressure, visual disturbances, retarded growth and diabetes insipidus.

Arise from neural ectoderm and epithelial elements in Rathke's pouch which is an embryonal outpouching in the floor of the third ventricle. These tumors can be located anywhere in the primitive craniopharyngeal duct that goes from the floor of third ventricle into the body of sphenoid bone and retropharyngeal space. It accounts for 7% of children's brain tumors, and 50% of tumors around the sella. Ninety percent are suprasellar and 10% are intrasellar. Benign and slow growing.

Typically nests or cords of stratified squamous or columnar epithelium present in the tumor. May be cystic, with a high cholesterol content.

Imaging Findings:
Large suprasellar mass with 60-80% having a thin and curvilinear calcification around the edge of the tumor.

On MRI, Can be bright on T1WI and T2WI if it is cystic with a high cholesterol content or if it has methemoglobin within in. Low signal intensity on T1WI and T2WI can be due to cystic lesions high in keratin content or tumors with extensive calcification.

Suprasellar masses

See References Chapter.

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