by Dr Noreen Norfaraheen Lee Abdullah MD (UKM), MMed Radiology (UKM), AM(Mal)

Answer to Case Of the Month 
Craniopharyngioma

Discussion of Findings
There is a large heterogenously enhancing mass arising from the intrasellar region extending upwards to the suprasellar region and to the anterior cranial fossa, partially compressing on the third ventricle. The mass consists of a central hypodense area. Specks of calcification are seen within. Both lateral, third and fourth ventricles are dilated. Periventricular lucencies around the lateral ventricles are seen and in keeping with acute hydrocephalus. The brain parenchyma of both cerebral hemispheres is compressed with effacement of sulci and basal cisterns.

Craniopharyngioma accounts for 6 to 7 percent of all intracranial tumours of childhood. The tumour is located intrasellar, suprasellar or both. Frequently they compress and displace the optic chiasm. Hypothalamus and the third ventricle being in close proximity may be involved as well. Thus the presenting symptoms range from increased intracranial pressure, growth retardation, visual disturbance to diabetes insipidus.

Initial investigation should include a skull radiograph. Features that will be prominent will be sella turcica erosion and enlargement. Calcification is frequently seen and the pattern may be flocculent, curvilinear, coalescent or a mixture. CT brain both plain and contrast enhanced scans are helpful. It demonstrates the sellar mass well, usually large and calcified. Most of the time a low-density area within the tumour is present. The Hounsfield units are that of water, sometimes, fat density. The tumour is not very vascular; this explains the enhancement as usually mild/moderate. The tumour may extend into the anterior and middle cranial fossa and cause displacement of the vessels. Occlusion of the Foramen of Monro will result in hydrocephalus. Magnetic Resonance Imaging is accurate in demonstrating the origin and extent of the mass. The signal intensity is variable on T1 and T2-weighted images. MRI is excellent in differentiating the tumour contents ie. blood, fat, debris, water or calcification.

Differential diagnosis considered besides craniopharyngioma are Rathke’s cleft cyst, hypothalamic glioma, metastases, meningioma, optic glioma, suprasellar germinoma and hamartoma.

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Last Updated:
Sunday, 17 August 2008