Introduction: Choroid plexus carcinoma (CPC) is a WHO grade III primary brain tumor more commonly found in the pediatric population and carries a poor prognosis. Maximal surgical resection is standard of care, however the role of adjuvant therapy remains controversial. We performed a meta-analysis to determine if adjuvant therapy improves tumor control and overall survival.

Methods: We performed an English language literature search in the PubMed database using the keywords “choroid plexus carcinoma” and created a meta-analysis database containing 152 individual patients who underwent surgical resection and for whom disaggregated clinical information is available, including age, extent of resection, adjuvant therapy, and overall survival.

Results: Age, extent of resection, and adjuvant therapy were all strongly predictive of overall survival using multivariate cox regression. Compared to patients who had surgery alone, those receiving chemotherapy or combined chemo- and radiotherapy had significantly improved overall survival( p<0.01), while adjuvant radiotherapy did not (p = 0.127) in the same cox model. Similar results were obtained from the subgroup of patients who had subtotal resection. However, by Kaplan-Meier analysis, adjuvant radiotherapy was still more beneficial than surgery alone ( p<0.05). Moreover, cox regression showed that combined adjuvant therapy improves survival compared to radiotherapy alone, but not chemotherapy alone. Finally, multivariate binary logistic regression showed that the use of any form of adjuvant therapy is correlated with significantly improved tumor control at 12 months (p<0.01), but only the use of combined adjuvant therapy improved tumor control at 5 years (p<0.05).

Conclusions: Adjuvant chemotherapy used alone or with radiotherapy can improve overall survival for patients with CPC. Tumor control at up to 5 years can be best achieved with combined adjuvant therapy. More data is needed for further characterize the role of adjuvant radiotherapy and identify specific chemotherapy agents most effective for tumor control and improving survival.

Patient Care: Due to the lack of strong and sufficient evidence, there is no currently consensus on the use of adjuvant therapy for the treatment of choroid plexus carcinoma. Our meta-analysis data strongly advocates the use of adjuvant therapy not only for tumor control but also to extend overall survival. Additionally, our data shows that chemotherapy can be much more effective than radiotherapy to extend overall survival, but combined adjuvant therapy can best increase tumor control at up to 5 years. Overall, our data may help to shape guidelines for treating choroid plexus carcinoma at individual institutions.

Learning Objectives: Choroid plexus carcinoma is a malignant primary brain tumor with poor prognosis. Maximum surgical resection is recommended, but the use of adjuvant therapy has not been standardized. Our data show that adjuvant chemotherapy strongly improves overall survival, but radiotherapy without chemotherapy may not. Moreover, combined adjuvant therapy can help to achieve the best tumor control

References: Gopal, P., J. R. Parker, et al. (2008). "Choroid plexus carcinoma." Arch Pathol Lab Med 132(8): 1350-1354.