Adamantinomatous craniopharyngioma

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Decreased vision.

Patient Data

Age: 30 years
Gender: Male

Solid and cystic mass with internal calcification is seen centered over the sella and suprasellar regions with extension posteriorly through the left aspect of the dorsum sellae and petrous apex. The involved portions of these bones are scalloped at margins.

There is partial compression of the third ventricle. No hydrocephalus. No intracranial hemorrhage was identified. Elsewhere grey-white matter differentiation is preserved.

There is a heterogeneous mass lesion with involvement of the sella and suprasellar region as well as extent along the dorsal aspect of the clivus.

The mass demonstrates solid, heterogeneously enhancing components, cystic components and regions of calcification, however, there are no regions of acute hemorrhage within the tumor. There is expansion of the sella.

Possible tumor infiltration into the right cavernous sinus is noted, however, normal flow voids are preserved within the cavernous segments of bilateral internal carotid arteries.

The mass displaces and compresses on the optic nerves bilaterally (left more than right).

The left optic nerve is draped along the superior margin of the suprasellar extent of the mass with marked flattening of the cisternal and prechiasmatic optic nerve segments.

The right optic nerve courses along the right lateral margin of the tumor with less overall distortion. The optic chiasm is draped along the superior margin of the mass and is markedly flattened.

The A1 and A2 segments of the anterior cerebral arteries are in close proximity to the anterior and lateral margins of the suprasellar tumor extent. The tumor impresses on the floor of the third ventricle, however, there is no hydrocephalus. The retroclival extent of the tumor causes mild impression on the pons and basilar artery.

No remote intra or extra-axial mass lesions or acute collections. No acute infarcts.

Conclusion:

Imaging appearances are in keeping with a large sellar and suprasellar craniopharyngioma causing flattening and distortion of the optic nerves (left more than right) and optic chiasm.

Case Discussion

The patient went on to have a resection. 

Histology

MICROSCOPIC DESCRIPTION:

Paraffin sections show normal anterior pituitary tissue composed of acini with a mixed cell population. At one edge there is a rim of loose fibrous tissue containing irregular trabeculae of calcium and collections of ghosts of effete squamous epithelial cells consistent with craniopharyngioma. No intact squamous epithelium is identified.

The sections show fragments of tissue in which there are irregularly shaped islands of epithelium. These have a peripheral basaloid layer, central squamous differentiation and an intermediate zone of loose stellate reticulum. Multifocal calcification is noted and there are collections of ghosts of effete squamous epithelial cells within hyalinised fibrous tissue. The features are of craniopharyngioma.

Tumor is seen to extend between trabeculae of viable bone. No adjacent brain parenchyma is included.

FINAL DIAGNOSIS:

Adamantinomatous craniopharyngioma.

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