Choroid Plexus Tumors in Children: A Population-Based Study

Abstract

Background: Choroid plexus tumors are rare neoplasms that primarily occur in children. The use of the SEER (Surveillance, Epidemiology and End Results) database allows for the analysis of the relationship between prognostic factors and survival. Methods: We analyzed the SEER database to select pediatric patients (<18 years old) with histologically confirmed diagnoses of choroid plexus papillomas (CPP; WHO Grade 0), atypical CPP (WHO Grade I) and choroid plexus carcinomas (CPC; WHO grade III). In univariate and multivariate analysis, we analyzed the relationship between demographic (age, gender, race, date of diagnosis) and treatment factors (extent of surgical resection, use of adjuvant radiation) on survival. Results: Overall, 168 pediatric subjects with choroid plexus tumors were identified as follows: 75 cases of CPP, 12 cases of atypical CPP and 81 cases of CPC. The median follow-up time was 3.5 years for CPP and 7.7 years for CPC. The median age at diagnosis was 4 years for CPP (10-90th percentile 0-16 years) and 1 year for CPC (10-90th percentile 0-10 years). In univariate regression analysis, CPC histology (β = -3.2, 95% confidence interval, CI -4.8 to -1.5, p < 0.001) was significantly associated with younger age at diagnosis in comparison to CPP. The mean tumor size was 3.7 cm for CPP and 6.0 cm for CPC (p < 0.001). A higher-grade tumor was associated with significantly increased mortality (hazard ratio, HR = 28.90, 95% CI 3.94-211.83, p = 0.001). Overall survival at 5 years was 98.7% for CPP and 58.5% for CPC (p < 0.001). Among those patients with CPC, gross total resection (GTR) was associated with a significantly lower mortality (HR = 0.21, 95% CI 0.07-0.66, p = 0.007). Overall survival at 5 years was 70.9% after GTR, significantly better than 35.9% after subtotal resection (p = 0.012) and 30% after no surgery (p = 0.003). Radiation treatment was not found to confer a survival benefit in CPC. No demographic characteristics (age, sex, race, date of diagnosis) were significantly associated with mortality. Conclusions: Analysis of a pediatric cohort of choroid plexus tumors in children in the SEER database shows that tumor grade is predictive of survival. In cases of CPC, the extent of surgical resection, especially GTR, is significantly associated with increased survival. Radiation did not confer survival benefit.

Myc and Loss of p53 Cooperate to Drive Formation of Choroid Plexus Carcinoma

“…CPCs, in contrast, usually require surgical removal followed by radiation and chemotherapy. Despite aggressive treatments, the 5-year overall survival rate is less than 60% (1,2) and the median progression-free survival (PFS) is only 13 months (3). Patients who do survive often suffer devastating side effects from the therapy, including neurocognitive deficits, endocrine disorders, and secondary cancers.…”

Pediatric choroid plexus carcinoma: Biologically and clinically in need of new perspectives

“…Choroid plexus (CP) carcinomas are rare malignant central nervous system (CNS) tumors derived from CP epithelium which account for less than 1% of all CNS tumors and up to 40% of CP tumors . The median age at diagnosis of patients with CP carcinoma is 1 year with more than 75% diagnosed before the age of five . Sixty‐five percent of CP carcinomas occur in males.…”

“…1 The median age at diagnosis of patients with CP carcinoma is 1 year with more than 75% diagnosed before the age of five. 2 Sixty-five percent of CP carcinomas occur in males.…”

Incidental choroid plexus papilloma in a child: A difficult decision

“…As a small percentage of these may be high grade, it is worth the risk to identify this patient earlier, especially because the extent of resection is significantly associated with increased survival. [4] On 1-year follow-up, our patient is doing quite well as she is reaching normal growth and developmental milestones. A 1-year MRI [Figure 4] was unremarkable for any residual or abnormal enhancement except postsurgical changes.…”