Craniopharyngioma (adamantinomatous)

Case contributed by RMH Report Writing

Presentation

Worsing vision.

Patient Data

Age: 35 years
Gender: Male
X-ray

Skull X-rays

There is no expansion or widening of the sella. The remainder skull is unremarkable. 

CT

CT Brain

There is a suprasellar mass with peripheral calcification. The central component of the mass is of low density (HU 15). The pituitary is of normal size and seen separate to the mass.

No erosion or remodelling of the bone adjacent to the mass and no extension into the sphenoid sinus. Mass abuts the basilar artery and distorts the optic chiasm.

No hydrocephalus. The remainder of the brain is unremarkable. Mucosal polyp within the left maxillary sinus. Nasal meati are patent with no significant septal deviation. Sphenoid sinus is well aerated. Anterior clinoid processes are non-aerated.

Conclusion: Suprasellar mass most likely represents a craniopharyngioma although correlation with MRI images is recommended.

MRI

MRI Brain (targeted to pituitary gland/sella)

Lobulated cystic mass is centred in the posterior suprasellar region with marginal T1 hyperintensity correlating with calcification seen on CT.

There is irregular enhancement of the periphery of the cysts.

The lesion is separate from the normal-appearing pituitary tissue but is inseparable from the infundibulum which seems to pass from anterior toward the right side of the mass.

The mass abuts the posterior margin of the optic chiasm, splaying and laterally displacing the optic tracts.

It indents the floor of the third ventricle and contacts without compressing or encasing supraclinoid ICAs, ACAs and basilar artery. No pulsation artefact seen in relation to this mass. The remainder of the imaged brain (please note this study has been targeted to the pituitary, and as such the whole brain has not been imaged) appears unremarkable.

Conclusion: Findings are most consistent with a suprasellar adamantinomatous craniopharyngioma.

Case Discussion

The imaging appearances of this case are highly suspicious for an adamantinomatous craniopharyngioma. Differentials for similar masses in this location, much less likely in this case,

  • Rathke cleft cyst
    • no solid or enhancing component
    • calcification is rare
    • unilocular
    • the majority are completely or mostly intrasellar 
  • pituitary macroadenoma (with cystic degeneration or necrosis)
    • can look very similar
    • usually has intrasellar epicentre with pituitary fossa enlargement rather than the suprasellar epicentre
    • despite occasional presence of T1 bright cystic regions, calcification in these cases is often absent (whereas most adamantinomatous craniopharyngiomas are calcified)
  • intracranial teratoma
    • presence of fat is helpful but requires fat saturated sequences or CT to confirm

 

MICROSCOPIC DESCRIPTION: Paraffin sections show several fragments of an adamantinomatous craniopharyngioma. These consist of irregularly shaped islands of epithelium with prominent peripheral basaloid cell layers, central squamous epithelium with focal keratinization and intermediate stellate reticulum. No mitotic figures or areas of necrosis are identified. Adjacent brain parenchyma shows well-developed piloid gliosis with numerous Rosenthal fibres identified.

DIAGNOSIS:  Sellar region lesion: Adamantinomatous craniopharyngioma.

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Case information

rID: 42784
Case created: 8th Feb 2016
Last edited: 6th Nov 2017
Tag: rmh
Inclusion in quiz mode: Included

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