Choroid plexus tumors: A spectrum from benign to malignant

Tumor discovery Pub Date : 2023-08-18 DOI:10.36922/td.1057
Ali A Mohamed, Thomas Caussat, Sophie Kelly, Phillip M Johansen, Brandon Lucke-Wold
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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, immunohistochemistry, imaging, treatment, and prognosis, CPTs cannot be reliably distinguished based solely on clinical presentation. Therefore, in this review, we aim to provide a comprehensive overview of each tumor subtype, along with the current management approach and emerging treatments.
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脉络膜丛肿瘤:从良性到恶性的光谱
脉络膜丛肿瘤(CPT)被认为起源于脉络膜丛的生长。尽管cpt具有广泛的症状、侵袭性和预后,但由于其与脑室的关系,以及与其生长相关的机械阻塞和肿块效应,大多数cpt通常表现出相似的表现。此外,这些肿瘤主要影响儿童人群,进一步使良性和恶性亚型的区分复杂化。世界卫生组织根据有丝分裂活性将cts分为三级,即I级、II级或III级,这决定了肿瘤的良性或恶性性质。世界卫生组织(WHO)对脉络膜丛乳头状瘤(CPP)、非典型CPP (aCPP)和恶性脉络膜丛癌(CPC)进行了分类。脉络膜丛腺瘤是另一种未被WHO正式分类的良性cpt。尽管在组织学、免疫组织化学、影像学、治疗和预后方面存在差异,但cts不能仅根据临床表现进行可靠的区分。因此,在这篇综述中,我们的目标是提供每个肿瘤亚型的全面概述,以及当前的管理方法和新兴的治疗方法。
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