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Tumor Discovery

REVIEW ARTICLE
Choroid plexus tumors: A spectrum from benign

to malignant

1
Ali A. Mohamed , Thomas Caussat , Sophie Kelly , Phillip M. Johansen , and
2
1
1
Brandon Lucke-Wold *
3
1 Charles E. Schmidt College of Medicine, Florida Atlantic University, Boca Raton, Florida, USA
2 Department of Neurosurgery, University of South Florida, Orlando, Florida, USA
3 Department of Neurosurgery, University of Florida, Gainesville, Florida, USA

Abstract

Choroid plexus tumors (CPT) are believed to originate from outgrowths of the choroid
plexus. Despite their broad spectrum of symptoms, invasive nature, and prognosis,
most CPTs typically exhibit similar presentations due to their relationship with the
cerebral ventricles, as well as the mechanical obstruction and mass effect associated
with their growth. In addition, these tumors mainly affect the pediatric population,
further complicating the differentiation between benign and malignant subtypes.
The World Health Organization classifies CPTs into three grades, namely, grades I,
II, or III, based on their mitotic activity, which determine the benign or malignant
nature of the tumors. CPTs classified by the World Health Organization (WHO) include
choroid plexus papillomas (CPP), atypical CPPs (aCPP), and malignant choroid plexus
carcinomas (CPC). Choroid plexus adenomas represent an additional category of
benign CPTs not officially classified by the WHO. Despite the variations in histology,
*Corresponding author: immunohistochemistry, imaging, treatment, and prognosis, CPTs cannot be reliably
Brandon Lucke-Wold
(brandon.lucke-wold@ distinguished based solely on clinical presentation. Therefore, in this review, we aim
neurosurgery.ufl.edu) to provide a comprehensive overview of each tumor subtype, along with the current
management approach and emerging treatments.
Citation: Mohamed AA, Caussat T,
Kelly S, et al., 2023, Choroid plexus
tumors: A spectrum from benign to
malignant. Tumor Discov, Keywords: Choroid plexus; Choroid plexus tumors; Choroid plexus adenoma; Choroid
2(2): 1057. plexus papilloma; Choroid plexus carcinoma
https://doi.org/10.36922/td.1057
Received: June 8, 2023
Accepted: July 28, 2023 1. Introduction
Published Online: August 18, 2023
Choroid plexus tumors (CPT) encompass a spectrum of severity, ranging from
Copyright: © 2023 Author(s). asymptomatic and benign cases to highly aggressive and malignant cancers. They
This is an Open Access article
distributed under the terms of the constitute approximately 1 – 4% of childhood brain tumors and are commonly
Creative Commons Attribution observed within the 1 year of life, accounting for up to 20% of brain tumors in children
st
License, permitting distribution, and [1,2]
reproduction in any medium, which under the age of 1 year old . CPTs are graded from 1 to 3 as benign choroid plexus
provided that the original work is papilloma (CPP), atypical CPP (aCPP), and malignant choroid plexus carcinoma
properly cited. (CPC), respectively. In addition, rare cases of choroid plexus adenomas (CPA) have been
Publisher’s Note: AccScience reported, usually as incidental findings. Benign, grade I tumors in the fourth ventricle
Publishing remains neutral with and cerebellopontine angle are commonly observed in adults . In addition, CPTs in
[3]
regard to jurisdictional claims in [3]
published maps and institutional the cerebellopontine angle are more benign than those located in the fourth ventricle .
affiliations. In contrast, malignant, grade III tumors in the supratentorium are more prevalent in

Volume 2 Issue 2 (2023) 1 https://doi.org/10.36922/td.1057