Disseminating histologically benign multiple papilloma of the choroid plexus: case report

A rare case of extensively disseminating multiple benign choroid plexus papilloma is shown. The patient first reported with high-grade hydrocephalus and two tumours in the 3rd and 4th ventricle was treated by atrioventricular shunt insertion, subtotal resection of the 4th ventricle tumour, and adjuvant 60Co irradiation of the posterior fossa. The dissemination that followed was revealed by computerised tomography and magnetic resonance imaging, and involved both the supra- and infratentorial ventricular systems, spinal canal, and brain parenchyma. Three years after the resection of the 4th ventricle tumour, the patient underwent excision of a temporal lobe lesion for relief of neurological symptoms, but showed no improvement and died 5 years after the primary diagnosis of CNS tumour. An autopsy was not performed. Analysis of the primarily resected mass showed distinct papillary pattern with no anaplasia, mitoses, multinucleation orgiant cell formation, and cytokeratin positivity at the absence of vimentin and glial fibrillary acidic protein. Analysis ofthe temporal lobe tumour again showed definite papillary formation with no signs of malignisation and virtually no mitotic figures, and the presence of cytokeratin, but not vimentin or glial fibrillary acidic protein. On both occasions, the diagnosis was choroid plexus papilloma (WHO grade I).