Introduction: Choroid Plexus Papillomas (CPC) and Choroid Plexus Carcinomas (CPC) are uncommon tumors that primarily manifest in children. However, little is known about the epidemiology, survival and prognostic factors of these tumors in adults. We performed a comprehensive epidemiological analysis of CPPs and CPCs using the Surveillance, Epidemiology and End Results (SEER) database, which queried patients from 1973-2014.
Methods: We queried the SEER database for all cases of CPP and CPC in patients aged 20 and above. Patient demographic data, surgical procedures and survival data was obtained and analyzed. Outcomes were assessed using univariate models, Kaplan-Meier survival curves, and Cox proportional-hazard regression analysis.
Results: A total of 256 cases were identified: 216 CPP cases and 40 CPC cases. The proportion of males with CPP and CPC were 36.6% and 42.5%, respectively (p=0.594). Age of diagnosis, race, sex and tumor location were not correlated with the incidence of CPP versus CPC. Cause specific mortality was 0% and 40% for CPP and CPC respectively. Overall mortality in the CPC cohort was significantly higher than the CPP cohort (Odds Ratio [OR] 8.388, 95% Confidence Interval [CI] 3.759809-18.710339, p<0.001). Age of diagnosis (Hazard Ratio [HR] 1.05, 95% CI 1.03-1.07, p < 0.001), Male sex (HR 3.17, 95% CI 1.75-5.79, p<0.001) and a CPC histology (HR 2.66, 95% CI 1.25-6.05, p = 0.014) were the only significant predictors of a worse overall survival amongst all patients.
Conclusions: Adult Choroid Plexus Tumors have a significantly different epidemiology compared to pediatric cases. While adult CPCs and CPPs have a higher incidence in women, both confer a worse prognosis in males. Further studies perhaps aimed at better elucidating the genetic cause of these tumors could clarify this disparity.
Patient Care: Choroid plexus tumors are very rare - especially in adults. As such, there has been very little research done on the epidemiology of these tumors, with studies being mostly restricted to case series. To our knowledge, this is the first population based study investigating the epidemiology and outcomes of this tumor specifically in the adult population. This study will provide clinicians with epidemiological and prognostic information to communicate to patients who are diagnosed with this tumor. Furthermore, it will encourage further investigation into sex differences and tumor survival.
Learning Objectives: 1) To understand the epidemiology of Adult Choroid Plexus Papillomas and Carcinomas using the Surveillance, Epidemiology and End Results (SEER) Database
2) Describe the actuarial rate of survival associated with the tumors
3) To understand factors associated with survival of patients
References: Cannon DM, Mohindra P, Gondi V, Kruser TJ, Kozak KR. "Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management". J Neurooncol. 2015; 121(1):151-7