Page 44 - TD-2-2

Tumor Discovery Choroid plexus tumors: Benign to malignant

pediatric populations . The 5-year survival rates for CPT acini. Mitotic activity, necrosis, and nuclear pleomorphism
[4]
patients vary, ranging from 81% in those diagnosed with are typically absent on microscopy, and the basement
CPP to 41% in patients with CPC . Poor prognosis in membrane is continuous and covered by cuboidal cells [14,21] .
[3]
CPTs is associated with age >40 or <10 . Immunohistochemistry results revealed that CPAs were
[3]
CPTs are often asymptomatic and typically positive for cytokeratin, S-100, transthyretin, vimentin,
manifest clinically only after significant tumor growth and GFAP in one case. Reports vary on the expressivity of
and malignancy development. CPTs can cause cell adhesion molecule (CAM) 5.2 and cytokeratins 7 and
[9,14,21]
mechanical obstruction of cerebrospinal fluid (CSF) 20 . Similarly, reports vary on calcifications, with one
flow, blockage of arachnoid granulation due to tumor case reporting calcifications and piloid astrocytosis with
hemorrhage, and overproduction of CSF, resulting in Rosenthal fibers [9,14] . CPAs have been reported to originate
hydrocephalus and subsequent symptoms such as headache, in the lateral and fourth ventricles [10,13,16,17,20,24] .
diplopia, and ataxia . Commonly used, yet non-specific, The typical clinical presentation of CPAs is
[5]
biomarkers for CPT diagnosis include transthyretin, glial hydrocephalus, and imaging techniques such as
fibrillary acidic protein (GFAP), epithelial membrane computerized tomography (CT) and magnetic resonance
antigen, and synaptophysin . Lateral ventricle CPP, one imaging (MRI) may reveal a calcified mass with solid
[6]
of the more common types of CPTs, often appears as a and/or cystic components. Ventricular enlargement may
hypertrophied anterior choroidal artery on imaging. In not be present, but perfusion MRI will typically reveal
addition, symmetrical hydrocephalus observed on imaging increased cerebral perfusion [9-21] . The treatment approach
may also aid in the diagnosis of CPTs . for CPAs is surgical resection. Good outcomes have been
[7]
An early intervention involving tumor resection, reported with gross total resection, multistage resection,
coupled with adjuvant treatments such as chemotherapy or subtotal resection, and biopsy [9,10,21] .
radiotherapy, is the standard of care for CPTs, as metastasis 2.2. Choroid plexus papilloma
is associated with poor outcomes. Ventriculoperitoneal
(VP) shunts are beneficial both pre- and post-operatively CPPs are benign neoplasms of the central nervous system
to prevent cerebellar tonsil herniation and address acute with a neuroepithelial origin, proposed to represent
[25]
hydrocephalus. The exact treatment approach varies hamartomatous overgrowths . These rare tumors are
[26]
depending on tumor grade, size, and location. However, most commonly present in the first 10 years of life .
surgical resection combined with adjuvant treatment CPPs account for <1% of brain tumors in adults, 2.3%
remains crucial for attaining optimal outcomes . Tumor of primary intracranial tumors in children, and 3.9% of
[8]
relapse is associated with worse outcomes, particularly in cerebral neoplasms in infants [22,24,27,28] . The previous studies
[3]
patients with CPC . have demonstrated associations between the occurrence of
CPTs and the BK virus, the John Cunningham (JC) virus,
This review focuses on novel approaches for diagnosing and the Simian virus (SV) 40 [29,30] . Other studies have found
and treating CPTs. In addition, differences between benign associations with tumor suppressor 53 (TP53) germline
and malignant CPTs and their locations will be explored mutations, Pierpont syndrome, Aicardi syndrome, 9p
to further explain variations in treatment approaches. This duplication, and hypomelanosis [31-34] . For instance, one
study will provide a comprehensive presentation by delving study identified the R248W mutation of the TP53 gene as
into differences in pathophysiology, prevalence, treatment, a common defect in patients with CPTs . Despite these
[35]
and prognosis of benign and malignant CPTs. associations, the etiology of CPPs remains unclear.
2. Choroid plexus tumors with benign The WHO classifies CPPs as grade I CPTs with <2 mitotic
pathology figures per 10 high power fields (HPFs) [27,36] . Mitotic activity,
necrosis, and nuclear pleomorphism are typically absent in
2.1. Choroid plexus adenomas microscopy. However, bland columnar epithelium can be
[37]
CPAs are benign CPTs that have been infrequently observed lining papillary fronds . Grossly, CPPs are pink,
described in existing literature . These tumors are friable, soft, globular with irregular projections, and highly
[9]
rare, with only 13 previous cases being reported, found vascular. Immunohistochemistry analysis has revealed
across a broad age range, including 5 cases in the that CPPs are positive for cytokeratin, podoplanin, S-100,
pediatric population and 8 cases in adults [9-21] . The World and vimentin [37,38] . CPPs may show positive expression for
Health Organization (WHO) does not have a separate GFAP, with higher expressivity observed in patients above
classification for CPAs [22,23] . Histologically, CPAs appear as 20 years of age. Transthyretin follows a similar trend with
well-differentiated tubular glands and irregularly shaped higher expressivity in older patients with CPPs . S-100
[9]

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