Choroid plexus tumors in adult and pediatric populations: the Cleveland Clinic and University Hospitals experience

Abstract

Choroid plexus tumors (CPT) are rare neoplasms accounting for 1-4% of all pediatric brain tumors. They are divided into choroid plexus papilloma (CPP), atypical choroid plexus papilloma (APP) and choroid plexus carcinoma (CPC). CPTs are known to primarily affect children less than 2 years of age. Gross total resection is the most important predictor of survival especially in CPC. Although small case series have been published, limited clinical data are available to describe treatment and outcome of CPTs. More clinical data would be necessary to complete the picture, particularly in populations that are not age limited. Here we share data from the two major hospitals in Cleveland to describe treatment and outcome of adult and pediatric patients. We performed a retrospective analysis of patients with CPT seen in Cleveland Clinic from 1990 to 2015 and at University Hospitals from 1994 to 2015. Results were compared to previously published historical controls. We identified 30 cases with CPT, including 22 pediatric and eight adult cases; 11 females and 19 males. The mean age at presentation was 12.4 years with a median age of 4.5 years (range 2 months-51 years). Gross total surgical resection was achieved in 22, subtotal resection in four, partial resection in two and unknown in two. The histology was CPP in 23 patients, two of whom developed recurrence requiring repeat resection and adjuvant therapy. Median event free survival (EFS) for CPP patients was 7.6 years. The histology was CPC in seven patients. All CPC patients were treated with adjuvant therapy. Median EFS of CPC patients was 4.4 years. Overall survival of all CPT patients was 100% with a median follow up of 7 years. A systematic literature review identified 1012 CPT patients treated from 1989 to 2013. The mean and median age of CPT patients was 13 and 3 years respectively. The median survival of 541 CPP patients was undefined vs. 2.7 years for the 452 CPC patients. The difference between the two populations was highly significant (p < 0.001). Kaplan-Meier survival curves comparing CPTs at Cleveland Clinic and University Hospitals versus a systematic literature review showed a statistically significant advancement in overall survival among the patients treated at Cleveland Clinic and University Hospitals. Our data are consistent with the literature review regarding epidemiology, clinical presentation, and treatment modalities but differed in regards to survival. Differences in survival may be related to different methods of data collection or details in patient care.

Ascites: a loadstar for the diagnosis and management of an intracranial tumor

“…5,6 Choroid plexus tumor of the central nervous system, a spectrum disorder based on tumor grading, covers CP hyperplasia, CP papilloma, and CP carcinoma. 7 Choroid plexus papillomas are benign tumors located mostly in lateral ventricles and that can be cured by surgical resection. 8 They mainly present in the first 10 years of life, especially in the first two years, with signs of increased intracranial pressure and hydrocephalus.…”

“…8 They mainly present in the first 10 years of life, especially in the first two years, with signs of increased intracranial pressure and hydrocephalus. 7 Choroid plexus is made of tufts of villi producing 100-150 mL of CSF daily in healthy adults and nearly half of that volume in children. 4 Besides, CP papilloma produces huge amount of CSF such as 400-5000 mL daily.…”

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

“…In humans, the most common location is the lateral ventricle in children and the fourth ventricle in adults ( 7 – 9 ). They account for 1–20% of brain tumors in children but only 0.5% in adults ( 10 , 11 ). In dogs, CPT is observed frequently in the fourth ventricle, however, it may also appear in the third and lateral ventricles ( 1 ).…”

“…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 ).…”

Rapid malignant progression of an intraparenchymal choroid plexus papillomas

“…CPTs account for only 0.3–0.6% of all brain tumors in adults and 2–5% in children. [ 1 2 3 19 ] The World Health Organization (WHO) classification describes three histological grades: grade I is choroid plexus papilloma (CPP), grade II is atypical papilloma, and grade III is the malignant form of carcinoma. [ 5 ]…”