Diffuse arachnoidal enhancement of a well differentiated choroid plexus papilloma

The case of a 38-year old man with a histologically benign choroid plexus papilloma arising within the fourth ventricle with en plaque growth around the brain stem and medulla is described in detail. Up to this point this particular growth pattern has not been published and is a rare presentation for this tumour.

The patient presented with a 1.5 year history of headache, nausea, and vomiting in the morning. Additional symptoms like blurred vision and gait ataxia lead to hospital admission. MRI demonstrated a homogeneously contrast-enhancing tumour completely filling the fourth ventricle and subsequent obstructive hydrocephalus. In addition Gd enhancement encasing the brain stem, the lower aspect of the medulla and the conus medullaris was seen suggesting a disseminated ependymoma or medulloblastoma.

An extensive resection of the tumour in the fourth ventricle and CP angle was performed. Infiltrative growth into the structures of the left CP angle and into the rhomboid fossa hampered complete removal. Surprisingly histological examination revealed a well-differentiated papillary choroid plexus papilloma without signs of anaplasia. On follow up imaging the Gd enhancement encasing the pons vanished completely. A growing cyst adjacent to a small tumour residuum left behind on the floor of the fourth ventricle led to re-operation after 8 months with complete removal.

This case presents several biological, neuroradiological and surgical aspects which make it noteworthy and we hope that the informations provided add to the understanding of these tumours, expand the differential diagnostic thinking of lesions which present with diffuse arachnoid Gd enhancement upon first presentation.