Craniopharyngioma - adamantinomatous

Case contributed by Dr Aisin Ibrahim

Presentation

Patient presented with increased forgetfulness, feeling of being slow to respond and decreased speed in mobility.

Patient Data

Age: 65 years
Gender: Female

Non-enhanced CT demonstrating a mass lesion in the suprasellar cistern region extending to the anterior region of the third ventricle. The lesion is mixed in density. The inferior aspect of the lesion is linear and calcified while the superior aspect the lesion is globular, well defined and spontaneously hyperdense.

The superior aspect of the lesion involves the anterior aspect of the third ventricle causing secondary obstruction at the level of the foramen of Monroe. The lateral ventricles are moderately dilated and there is periventricular white matter hypodensity consistent with transependymal edema, in keeping with acute hydrocephalus.  

MRI brain confirms the presence of a partially calcified suprasellar lesion extending in the anterior portion of the third ventricle and interpeduncular cistern.

The superior aspect of the lesion is round and well-circumscribed, with a hyperintense T1 signal and an intermediate high T2 signal, in keeping with a cystic/proteinaceous component. The inferior aspect of the lesion is more irregular, demonstrating both enhancing solid component and coarse calcifications.

The lesion is associated with obstructive hydrocephalus, with dilatation of the bilateral lateral ventricles due to obstruction of the interventricular foramen and evidence of periventricular transependymal CSF flow.

Histology

Sections reveal trabecular bone with the fibrotic bone marrow mostly with “wet” keratin debris. There is also wet keratin associated with marked piloid gliosis and small fragments of normal-appearing anterior pituitary. There is no viable neoplastic epithelium identified. 

Conclusion: Findings are compatible with craniopharyngioma, adamantinomatous type. 

Case Discussion

This 65-year-old patient presenting to the emergency room for cognitive deficits including increased forgetfulness and decreased speed in speech and mobility for the past few days.

The patient was initially thought to present symptoms of cerebrovascular accident and underwent initial CT head that demonstrated a mixed density partially calcified suprasellar mass lesion extending to the anterior third ventricle causing obstruction of the foramen of Monroe and secondary hydrocephalus. MRI confirmed the presence of a mass lesion and the pre-operative main diagnosis was a craniopharyngioma.

This lesion also shared several characteristics with a colloid cyst, including high attenuation content on unenhanced CT and secondary obstruction with acute hydrocephalus. However, the presence of calcification and pattern of extension to the suprasellar cistern made this diagnosis less likely.  

The patient underwent surgical resection and pathology findings were compatible with an adamantinomatous type craniopharyngioma and post-surgical imaging showed immediate improvement of the acute hydrocephalus.

The case was contributed by Dr Raquel Delcarpio, Associate Professor of Radiology at McGill University.

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Case information

rID: 82833
Published: 9th Oct 2020
Last edited: 9th Oct 2020
Inclusion in quiz mode: Included
Institution: McGill University