Management of choroid plexus tumors—an institutional experience

Abstract

BackgroundChoroid 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.MethodsRetrospective 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.ResultsTwenty-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.ConclusionsWe 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.

Infratentorial choroid plexus tumors in children

“…We encountered a higher rate of infratentorial CPT location (75%) in our patient population over the last decade, which is contrary to the rates reported in prior studies and our own prior experiences [10,14,15]. We did not have any CPC in our cohort; however, the incidence of CPC among CPTs of all locations is approximately 11% in prior studies [2], and its infratentorial occurrence is exceedingly uncommon [16]. However, others reported anaplastic choroid plexus carcinoma is a tumor with a predilection for the posterior fossa of infants [17].…”

“…Choroid plexus tumors (CPTs) are rare intraventricular tumors of neuroectodermal origin. CPTs occur predominantly in the lateral ventricle in children in contrast with adults who have primarily infratentorial lesions [1,2]. Choroid plexus tumors are classified by the World Health Organization (WHO) as choroid plexus papillomas (CPP) (WHO grade 1), atypical choroid plexus papillomas (aCPP) (WHO grade 2), and choroid plexus carcinomas (CPC) (WHO grade 3) [3].…”

Transcallosal Removal of a Choroid Plexus Tumor From the Lateral Ventricle in a Dog. Case Report

“…As in humans, the main intraoperative complication in our case was bleeding from the CPT ( 8 ). It was controlled with thermocoagulation and specific anticoagulation materials ( 8 , 19 ). Observation is most critical in the 24 h following surgery, especially in cases of intraventricular tumor removal ( 12 ).…”

“…According to the human data, total resection is the most effective treatment for CPTs, but it is challenging due to the major surgical risk caused by these tumors' high vascularity ( 11 , 19 , 29 ). The efficacy of the surgical removal of a CPT can be considered as the single most determinant factor for long-term survival ( 19 ). In one human study, tumor recurrence was observed in 25% of patients ( 19 ).…”

“…According to the 2007 and 2016 World Health Organization (WHO) classification of tumors of the central nervous system, CPTs are classified as either CPPs (WHO grade I), atypical CPPs (aCPPs) (WHO grade II), or choroid plexus carcinomas (CPCs) (WHO grade III) ( 4 , 5 , 9 , 10 , 13 , 19 , 20 ). Using the WHO classification system to categorize human CPTs, it could be verified that the grade of a tumor can be considered as the most important postsurgical prognostic factor ( 5 ).…”