Adamantinomatous craniopharyngioma

Case contributed by Dr Abiola Ayodele

Presentation

Impaired vision, headaches and head swelling.

Patient Data

Age: 5 years
Gender: Female

A large lobulated central cystic mass arising from the sella/parasellar region extending to the frontal lobe, more on the left. The mass is isointense on T1 to grey matter and hyperintense on T2/ FLAIR. It demonstrates peripheral enhancement on T1 post-contrast. Compressive mass effects on the pituitary gland, frontal cortex (with thinning), displacing the optic chiasm and infundibulum posteriorly, elevating the mamillary body and corpus callosum. The anterior horns of the lateral ventricles are effaced and the posterior horn of the right lateral ventricle is dilated.

There is mild perilesional edema. Also noted is transependymal edema around the lateral ventricles.

Case Discussion

Craniopharyngioma is commoner in the pediatric age group, though it has a bimodal distribution. Visual and pressure symptoms are very typical.  When tumors are large, they tend to extend in all directions with mass effects on the midbrain and optic chiasm.

This case is typical for adamantinomatous craniopharyngioma which was subsequently histologically proven.

Histology:

Microscopic examination shows foci of proliferating squamous cells bordered by palisading columnar epithelium. The cells bear ovoid nuclei with regular nuclear outlines and no striking mitotic figures. Dystrophic calcifications are seen dispersed within the tissue.

Diagnosis: adamantinomatous craniopharyngioma

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