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Adamantinomatous craniopharyngioma

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

VIsual disturbance.

Patient Data

Age: 40 years

Centered within the suprasellar region a predominantly cystic lesion is present with peripheral nodular components demonstrate enhancement on the post-contrast images. The pituitary fossa is not enlarged. This compresses the optic chiasm and displaces the third ventricle to the left. There is no hydrocephalus.

Conclusion: This most likely represents a cystic craniopharyngioma.

There is a heterogenous supra-sellar mixed solid-cystic mass lesion with a lobulated contour and a 'waist' formed by the constriction of the circle of Willis. Mineralization and enhancement is noted along the rim of the mass with more solidly enhancing regions along the base of the mass. The mass remodels dorsum sellae and extends posteriorly to the pre-pontine cistern, impressing on the ventral pons and basilar artery, however, normal flow voids are preserved within the basilar artery. Superiorly the mass splays the cerebral peduncles, causing compression of the right cerebral peduncle. The mass effaces the anterior aspect of the third ventricle, however, there is no hydrocephalus. The mass displaces the chiasm to the left and the right optic nerve inferiorly, flattening the nerve as well as the right side of the optic chiasm. Abnormal T2 signal change is demonstrated within the left side of the optic chiasm and along the proximal extent of the left pre-chiasmic optic nerve.The pituitary stalk is displaced left postero-laterally by the mass. No remote intra- or extra-axial mass lesions, acute hemorrhages or collections.

Conclusion: A large supra-sellar mass lesion is demonstrated with imaging features in keeping with a craniopharyngioma.

Case Discussion

The patient went on to have a resection. 

Histology

Paraffin sections show a partially cystic/partially solid tumor composed of irregularly shaped islands of epithelium. These have peripheral basaloid cell layers, central stellate reticulum and central keratinizing squamous epithelium. Focal dystrophic calcification is noted. The features are of adamantinomatous craniopharyngioma.

FINAL DIAGNOSIS: Adamantinomatous craniopharyngioma.

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