Papillary tumor of the pineal region

Case contributed by Ali Alsmair
Diagnosis almost certain

Presentation

The patient presented with headache.

Patient Data

Age: 45 years
Gender: Female

There is a rather well-defined lesion in the pineal region, measuring about 32 x 28 x 24 mm (AP.TRANS.CC), this lesion shows mixed central solid and peripheral cystic components, the central solid component shows diffusion restriction, with evidence of T1 hyperintensity in the anterior aspect of this lesion, which is not corresponding to fat, calcification or hemorrhage. This lesion seems to be inseparable from the tectum and midbrain and causes mass effect on them.

Peripherally located calcific foci and these lesions are noted.

After IV contrast administration this lesion showed heterogeneous enhancement of the solid component and the wall of the cystic component.

No evidence of hydrocephalus, with evidence of an enhancing tract extending from the frontal bone to the right lateral ventricle, representing sequela from a recent ventriculostomy.

Tiny avidly enhancing focus in the right frontal lobe is seen, which shows a blooming effect on SWI, compatible with a capillary telangiectasia. Incidental bilateral periventricular non-specific T2/FLAIR hyperintensities are noted, without mass effect or diffusion restriction.

An incidental well defined extra-axial cystic lesion in the posterior fossa is noted, measuring about 2 x 4 x 3 cm (AP.TRANS.CC), suggestive of posterior fossa arachnoid cyst.

The lesion appears as an isodense mass, with peripherally located calcifications. The T1 hyperintense region in the anterior part of the lesion appears isodense, without calcifications.

As the patient was diagnosed with a pineal mass, a ventriculostomy was performed to treat the hydrocephalus and for biopsy. The biopsy revealed papillary tumor of the pineal region.

Case Discussion

 The typical MRI findings of the papillary tumor of the pineal region show a well-circumscribed lesion with variable T1 signal intensity, high T2 signal intensity, and enhancement on postcontrast images. Cystic areas within the mass are commonly seen. Hyperintensity on T1-weighted images is sometime seen and is a specific finding, which is considered to be related to secretory inclusions containing glycoprotein or protein (not fat, calcifications or blood).1 2

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