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Adamantinomatous craniopharyngiomas

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Visual changes.

Patient Data

Age: 60 years
Gender: Female

There is a large mass centred on the suprasellar cistern, isointense to brain parenchyma on T1, hyperintense and heterogeneous on T2 with diffuse contrast enhancement, apart from a few small foci of non-enhancement, corresponding to well-defined areas of T2 hyperintensity.

The mass extends at least into the superior aspect of the sella. It is not entirely clear whether it is continuous with, or separate to the pituitary gland. There is the sense of an intensity difference particularly on T1- weighted imaging, between the mass and what appears to be a normal pituitary.

The bony sella is not enlarged. No convincing extension into either cavernous sinus.

Superiorly, the mass elevates and partially effaces the third ventricle. The hypothalamus and optic chiasm are elevated superiorly and not clearly seen. No hydrocephalus.

Conclusion: Large mass in the suprasellar cistern. While pituitary macroadenoma is by far the most likely cause for a mass in this region, normal bony sella, and possible separate normal pituitary gland suggest an alternative diagnosis of craniopharyngioma.

Case Discussion

The patient went on to have a resection. 

Histology

The paraffin-embedded sections confirm the intraoperative diagnosis of a craniopharyngioma. The tumour is composed of anastomosing sheets and nests of cells with prominent peripheral palisading columnar cells. The internal cells are loosely arranged and squamoid and there are scattered nodules of compact keratin. There are cysts containing homogenous pale blue staining material. There is focal necrosis and mild patchy chronic inflammation. There are no mitoses.

FINAL DIAGNOSIS: adamantinomatous craniopharyngioma

Discussion

This case is somewhat atypical as the mass is mostly solid without T1-high signal cysts or obvious calcification. At this age, adamantinomatous craniopharyngiomas are still somewhat more common than papillary craniopharyngiomas, although the incidence is similar. 

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