Metastatic atypical choroid plexus papilloma: a case report

Choroid plexus papillomas (CPPs) are rare adult tumours and metastatic disease is even less common, more typically associated with choroid plexus carcinoma. We present the case of a 32-year-old patient with multiple metastases arising along the length of the neuraxis 3 years after resection of an atypical fourth ventricular CPP. Metastatic deposits were found from the mid-brain to the lumbar cistern and the patient underwent repeat excision of the fourth ventricular tumour, partial resection of a cervico-thoracic deposit and craniospinal radiotherapy. Possible explanations for the rarity of atypical CPP include unclear diagnostic criteria leading to under-representation in reported cases. We review the current literature on metastatic CPP and discuss the role of surgery and adjuvant therapy in relation to both typical and atypical disease.