Management of choroid plexus tumors-an institutional experience

Abstract

Background: Choroid plexus tumors are rare entities. Resection is the mainstay of treatment in grade I and grade II tumors and adjuvant treatment is usually reserved for the less frequent choroid plexus carcinoma (CPC). Outcome is not only related to their histological grade but also dependent on their size, location, and presence of often multifactorial disturbances of cerebrospinal fluid (CSF) circulation.

Methods: Retrospective analysis of 36 consecutive patients operated on a choroid plexus tumor at our institution in a mixed pediatric and adult population between 1991 and 2016.

Results: Twenty-one CPP, 11 atypical choroid plexus papillomas (aCPP), and four CPC were encountered in 17 children and 19 adults. Regardless of histological grading, gross-total resection (GTR) could be achieved in 91.7% of patients. Tumor recurrence (25.0%) was significantly associated with histological grading (p = 0.004), subtotal resection (p = 0.002), and intraoperatively evident zones of tumor infiltration (p = 0.001). Adjuvant therapy was performed in 19.4% of patients, mainly diagnosed with CPC. The 5-year overall survival rate was 95.2% for CPP and 100.0% for both aCPP and CPC. Survival was related to the extent of resection (p = 0.001), tumor progression (p = 0.04), and the presence of leptomeningeal metastases (p = 0.002). Even after resection, either ventricular or subdural shunting was required in 25.0% of patients.

Conclusions: We could confirm that GTR is crucial for treatment of choroid plexus tumors. Parenchymal tumor infiltration as detected intraoperatively was associated with the extent of resection and not limited to CPC. CSF disturbances mandating treatment may persist after resection.

Keywords: Atypical choroid plexus papilloma; Choroid plexus carcinoma; Choroid plexus papilloma; Choroid plexus tumor; Gross-total resection; Infiltration.

Fig. 1

Kaplan–Meier plot displaying progression-free survival (a, b) and overall survival (c, d) in relation to histological tumor grade and extent of resection. aCPP, atypical choroid plexus papilloma; CPC, choroid plexus carcinoma; CPP, choroid plexus papilloma; GTR, gross-total resection; STR, subtotal resection

Fig. 2

Illustrative case no. 9. A 2-month-old girl presented with increased head circumference (> 97th percentile) and a contrast-enhanced intraventricular lesion at the Foramen of Monro (a). Using a transcallosal approach the tumor was totally resected (b) and histopathology diagnosed an atypical choroid plexus papilloma. Pre-operative hydrocephalus resolved, but a subduro-peritoneal shunt was necessary due to postoperative persistent subdural hygroma (c). Follow-up MRI scans revealed tumor recurrence 7 months after primary surgery (d), which was resected via a transcallosal approach. Long-term follow-up (6.7 years) showed complete remission and excellent neurological outcome

Fig. 3

Percentage of histological grading in children and adults

Fig. 4

Kaplan–Meier plot displaying progression-free survival (a) and overall survival (b) for children and adults