Craniopharyngioma (adamantinomatous)

Case contributed by Dr Rajalakshmi Ramesh

Presentation

Three month history of worsening headache, behavioral changes, blurred vision and diplopia. Right visual acuity measured a 6/15, and left eye only able to perceive light. Upper bilateral temporal field visual deficits. Full range of eye movements and bilaterally pale fundi. The remained of the cranial nerve and upper and lower limb examinations were unremarkable to sensorimotor testing.

Patient Data

Age: 9
Gender: Male

There is a predominantly cystic lesion occupying the sella and suprasellar region, measuring 36 x 21 x 21 mm (SI X AP X TR) in maximum dimensions. Peripheral rim of interrupted calcific specks are present surrounding this lesion. The lesion is predominantly hypodense with isodense contents along the inferior and left lateral wall of this lesion, which may represent solid components. It may represent a craniopharyngioma. The pituitary gland is not separately identified. The optic chiasm is anteriorly displaced and partially effaced by this lesion. No intraconal/extraconal lesion in either orbit. There is no extension of this mass into the third ventricle. Lateral, third and fourth ventricles are normal in size and configuration with no features of obstructive hydrocephalus. There is a small hypodense focus measuring 6mm in the left frontal lobe white matter, which is non-specific. Grey-white matter differentiation is preserved; extra-axial CSF spaces are within lower limits. Craniocervical junction is normal with no tonsilar herniation. No bony abnormality identified.

Sellar mass with suprasellar extension. Sella is enlarged, consistent with early/slow growing/longstanding lesion. Mass measures 2.3 x 2.2 x 3.8 cm (AP x TR x CC). Mixed signal intensity; cystic component with solid heterogeneously enhancing sella and left suprasellar component. Solid component has high T1 signal at right aspect with haemosiderin staining on gradient echo and restricted diffusion, all consistent with blood products. Solid component to left is low T1, heterogeneous T2 and enhances heterogeneously. Asymmetry of lateral ventricles with slightly dysplastic right but otherwise normal ventricular configuration and extra-axial spaces. Extra-orbital, prechiasmatic optic nerves, and chiasm are stretched and displaced around the superior aspect of the mass. Normal optic tracts and radiations. Displacement of the post-ophthalmic segments of the internal carotid arteries and anterior cerebral arteries around the mass. Normal flow voids. Taken together, this lesion is most consistent with craniopharyngioma.

A T2 hyperintense, non-enhancing focus in the left frontal lobe subcortical white matter follows CSF on all sequences except on FLAIR. It has a hyperintense rim, centrally isodense to grey matter on FLAIR. Lesion measures 7 x 5mm (ovoid). This is atypical for a perivascular space or gliotic focus. It raises the possibility of the sellar lesion being a germinoma (and so spinal imaging was performed, which failed to reveal any intraspinal lesion).

The patient underwent a right-sided pterional craniotomy and resection of the suprasellar mass.

Histology

In both specimens, there are sheets of epithelium with regular peripheral palisading of nuclei and a loosely reticular central arrangement. Numerous small cystic spaces are formed. The nuclei are uniform, lacking pleomorphism and mitoses are inconspicuous. Focally, there is dystrophic calcification. There is no evidence of necrosis.

Final Diagnosis:

Suprasellar Craniopharyngioma (adamantinomatous type).

 

Follow-up

Post-operatively, the patient’s recovery complicated by hypopituitarism, medically managed with thyroxine and hydrocortisone. His vision, however, improved markedly, returning to normal visual acuity (6/6 bilaterally) and normal visual fields. Follow-up imaging at the post-operative 8 week juncture demonstrated adequate debulking of the tumor, and less pronounced stretching and displacement of the optic chiasm and optic nerves.

A heterogeneous mass (craniopharygioma) is seen widening the sella and measuring 1.9 x 1.6 x 2.1 cm (AP X TR X CC), previously this tumor measured 2.3 x 2.2 x 3.8 cm. The mass has a cystic component on the left side which has reduced significantly compared to the previous pre-operative study. The solid component on the right enhances heterogeneously and demonstrates a thick ring-like pattern after contrast injection. Overall, there is a reduction in tumor size, after the resection. Thinning of the chiasm is seen particularly on the right, there is displacement of the post-opthalmic segments of the internal carotid arteries and anterior cerebral arteries around the mass; all these findings are less pronounced compared to the previous study. Normal optic tracts and optic radiations. The pituitary stalk is now seen and displaced posteriorly. Normal flow voids and normal opacification post contrast.

 

The T2 hyperintense, non-enhancing lesion in the left frontal lobe white matter follows csf on all sequences except FLAIR. It has a hyperintense rim, centrally isointense to grey mater on FLAIR. Lesion measures 7x5mm (ovoid). This is unchanged since the previous study.

 

Six months following his diagnosis, the patient represented with progressive visual decline in his left eye (to 6/7.5) and bilateral temporal field visual deficits, in conjunction with an Addisonian crisis and severe hyponatremia. Repeat imaging demonstrated recurrence of his craniopharyngioma with heterogenous enhancement in both the sella and suprasellar regions.

Comparison made with previous two studies. There has been regrowth of the previously debulked craniopharyngioma within the sella turcica and extending into the suprasellar region and now again abuts the central portion of the optic chiasm. Post debulking, the remaining largest posterior portion of the tumor measured 24mm (SI). On the current study, it measures 29mm (SI). The tumor complex is T2 hypointense and hyperintense within the sella and suprasellar region, and post-contrast shows heterogeneous enhancement with non-enhancing presumed cystic components or components with some calcification as seen on the original CT scan. Now, the tumor measures 29 x 18 x 20mm (whereas post-operatively this measured 19 x 16 x 21mm). There is asymmetrical mild dilatation of the lateral ventricles with the last right being slightly larger than the left. Normal third and fourth ventricles. The remainder of the brain parenchyma is normal.

The second lesion within the inferior frontal lobe subcortical white matter again follows CSF on all sequences except on FLAIR where it has a hyperintense rim of hypointense central component to grey matter. It is ovoid and measures 7mm in diameter.

The patient underwent an endoscopic transphenoidal debulking of the recurrent craniopharyngioma, followed by post-operative radiotherapy.

Histology

Paraffin sections show small fragments of craniopharyngioma. These consist of epithelial islands within a loose fibrous stroma. The epithelial islands show a peripheral basaloid layer, central squamous epithelium and a stellate reticulum layer.  Several cystic spaces filled with loose amorphous eosinophilic material are noted and there are several foci of dystrophic calcification.  No mitotic figures or areas of necrosis are identified.

Final diagnosis

Craniopharyngioma.

Case Discussion

This case illustrates the typical pathological and radiological features of adamantinomatous craniopharyngioma. 

 

Case courtesy of Dr. Frank Gaillard

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Case information

rID: 34580
Case created: 27th Feb 2015
Last edited: 7th Feb 2017
Inclusion in quiz mode: Included

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