Malignant progression of an oncocytic choroid plexus neoplasm in an adult patient with li-fraumeni syndrome: a case report

Abstract

Choroid plexus papillomas are uncommon, benign (WHO grade 1) tumours which most frequently arise in the lateral or fourth ventricle of young adults and children. Oncocytic change is rare in the central nervous system and its tumours, and only a few cases of the oncocytic variant of choroid plexus papilloma have been reported. We report an unusual case of a choroid plexus papilloma with oncocytic morphology, which recurred following subtotal resection and showed progression to an atypical papilloma. The tumour arose in a 33-year-old patient with a germline mutation of TP53 (Li-Fraumeni syndrome). Choroid plexus carcinomas are closely associated with Li-Fraumeni syndrome, and <i>TP53</i> mutations are predictive of an aggressive course. Oncocytic choroid plexus tumours are a rare variant which can present diagnostic difficulties and should be considered when faced with a CNS tumour composed of large, eosinophilic cells.