Adamantinomatous craniopharyngioma

Case contributed by Aisin Ibrahim
Diagnosis certain

Presentation

Increased forgetfulness, feeling of being slow to respond and decreased speed in mobility.

Patient Data

Age: 65 years
Gender: Female

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

The superior part of the lesion involves the anterior aspect of the third ventricle, causing secondary obstruction at the level of the foramen of Monro. 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 part 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 part 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 lateral ventricles due to obstruction of the interventricular foramen and evidence of periventricular transependymal CSF flow.

Case Discussion

A 65-year-old patient presented to the emergency room with cognitive deficits, including increased forgetfulness and decreased speed of 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 main preoperative diagnosis was a craniopharyngioma.

The 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 of the lesion.

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. 

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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