Adamantinomatous craniopharyngioma

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Visual disturbances.

Patient Data

Age: 8 years
Gender: Female

There is a large well-defined suprasellar solid-cystic mass with intrasellar extension. The solid component elicits a low signal on T1, slight high signal on FLAIR and T2 with heterogeneous enhancement on postcontrast sequences. The cystic component shows an isosignal to the cortical grey matter on T1 and very high signal on FLAIR and T2. No restricted diffusion is seen. A mass effect is noted on the optic chiasma which is laminated and displaced superiorly. The floor of the 3rd ventricle is slightly elevated with no ventricular dilatation.

Case Discussion

MRI features of a suprasellar solid-cystic mass compressing the optic chiasma, suggestive of a craniopharyngioma.

The patient went on to have a transsphenoidal resection and the histology confirmed the diagnosis of adamantinomatous craniopharyngioma.

Craniopharyngiomas are found in patients of all ages with however two picks. The first peak occurs between the ages of 5-15 years, consisting almost exclusively of the adamantinomatous subtype. A second smaller peak occurs in adults aged over 40 years old, consisting of both papillary and adamantinomatous subtypes. The papillary subtype is found almost exclusively in adults.

 

Additional contributor: R.Bouguelaa, MD

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