Treatment of choroid plexus tumors: a 20-year single institutional experience

Abstract

Object: Choroid plexus tumors (CPTs) are rare intracranial neoplasms that constitute approximately 2%-5% of all pediatric brain tumors. Most of these tumors present with severe hydrocephalus. The optimal perioperative management and oncological care remain a matter of debate. The authors present the epidemiological and clinical features of CPTs from a 20-year single-institutional experience.

Methods: A total of 39 consecutive patients with pathologically proven CPTs (31 choroid plexus papillomas [CPPs] and 8 choroid plexus carcinomas [CPCs]) were included in this series. Patient demographics, clinical presentation, comorbidities, indications for surgery, radiological studies, tumor location, and all operative variables were reviewed for each case. Multivariate regression analysis was performed to identify independent predictors of tumor recurrence and survival.

Results: The overall mean age (± SD) was 13.13 ± 19.59 years (15.27 ± 21.10 years in the CPP group and 3.66 ± 3.59 years in the CPC group). Hydrocephalus was noted at presentation in 34% of patients. The most common presenting symptoms were headache (32%) and nausea/vomiting (26%). Gross-total resection (GTR) was achieved in 86% of CPPs and in 71% of CPCs (p = 0.57). There was 100% survival in patients with CPPs observed at the 5- and 10-year follow-up and 71% survival in patients with CPCs at the 5-year follow-up. In a multivariate regression analysis, a diagnosis of papilloma, preoperative vision changes, or hydrocephalus; right ventricle tumor location; and GTR were all independently associated with a decreased likelihood of tumor recurrence at last follow-up.

Conclusions: The authors' study suggests that patients with CPCs are more likely to experience local recurrence and metastasis; hence, GTR with chemotherapy and radiotherapy, particularly for CPCs, is pivotal in preventing recurrence and prolonging survival. While GTR was important for local control following resection of CPPs, it had a minimal effect on prolonging survival in this patient cohort.

Fig. 1

Preoperative axial (left) and coronal (right) contrast-enhanced T1-weighted MRI studies showing a typical CPP arising in the posterior horn of the left lateral ventricle.

Fig. 2

Magnetic resonance images obtained in a 4-year-old girl, showing a representative CPC. A: Preoperative axial T1-weighted image demonstrating a CPC in the posterior horn of the left lateral ventricle. B: Postoperative axial T1-weighted image. C: Preoperative sagittal T1-weighted image showing a large carcinoma in the lateral ventricle. D: Complete excision was achieved. Postoperatively, the patient did not require VP shunt placement.

Fig. 3

Representative sagittal (left) and axial (right) T1-weighted contrast-enhanced MRI studies of a CPP arising in the fourth ventricle.

Fig. 4

Estimated Kaplan-Meier plot depicting survival after resection of CPPs and CPCs. Only patients with observation times > 0 were included. The median and IQR long-term follow-up of patients with CPPs was 21 months (IQR 4.5–104 months), while the median long-term follow-up of patients with CPCs was 71 months (IQR 40–117 months). Five- and 10-year survival for patients with CPPs was 100% compared with a 5-year survival of 71% for those with CPCs (5 of 7). The difference in survival for patients with CPPs versus CPCs was statistically significant (p < 0.01, log-rank test).