PURPOSE

The optimal treatment of patients with choroid plexus carcinoma (CPC) is unclear. We conducted a systematic review and meta-analysis of individual patient information to determine the effect of surgery, adjuvant therapy, and other prognostic factors for CPC.

METHODS AND MATERIALS

A comprehensive search of the PubMed and Google Scholar databases was performed using the following MeSH terms to find all possible reports on CPC: choroid plexus tumor; choroid plexus carcinoma; choroid plexus carcinoma AND treatment; and choroid plexus carcinoma AND survival. We performed an individual patient data analysis to assess the strength of the potential associations between different variables and the outcomes for patients with CPC.

RESULTS

Data from 284 patients were retrieved from 89 studies. The median patient age was 2 years, with 26% patients diagnosed in the first year of their life. Of these 284 patients, 52.8% had undergone gross total resection (GTR) or near total resection. The median follow-up period for the entire cohort was 10.8 months. The median progression-free survival (PFS) was 13 months (95% confidence interval 8.14-17.8). PFS was better for patients >aged 5 years and those who had undergone GTR. The median overall survival (OS) was 29 months (95% confidence interval 16.3-41.7). OS was better for patients aged >5 years, those who had undergone GTR, those who had received adjuvant treatment, and those with a parenchymal tumor site.

CONCLUSIONS

CPC is an aggressive tumor, with a median PFS of 13 months and median OS of 29 months. All patients should undergo maximal safe resection, because GTR is associated with improved survival. The use of adjuvant radiation and chemotherapy were also associated with improved outcomes.