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Tumor Discovery Choroid plexus tumors: Benign to malignant

Due to a lack of clinical and long-term follow-up CPTs because of the mechanical obstruction and mass
data, a standard treatment approach for aCPPs has not effect associated with the tumors and their relationship
been established . The suggested approach is maximal with cerebral ventricular anatomy [2,8,45,79] . Despite the
[70]
surgical resection , which often results in the resolution malignant nature of CPCs, clinical presentation alone is
[66]
of hydrocephalus, resolution of symptoms associated with not enough to distinguish them from other CPTs, as they
increased intracranial pressure, and a return to normal all present with similar symptoms. The MRI findings of
ventricular morphology in most cases. Hydrocephalus may CPCs are highly variable, demonstrating masses that are
persist in some cases even after treatment . Chemotherapy iso- or hypointense, hyper-, hypo-, or isointense, and
[68]
courses with and without irradiation administered in cases hypo-, iso-, hyper-, or mixed intensity on T1WI, T2WI,
of aCPPs with metastases and incomplete resection have and DWI, respectively [68,69,71,75,76,82] .
demonstrated an 89% 5-year overall survival probability .
[66]
The most effective proposed treatment for CPCs is
3. Choroid plexus tumors malignant a combination of surgical resection, radiotherapy, and
pathology chemotherapy. However, the order of treatment modality
and the required amount of adjuvant treatment remain
3.1. Choroid plexus carcinomas unclear and are debated in the literature [80,83] . Although
CPCs are a malignant neoplasm type of CPTs proposed surgical resection is the most important factor for long-term
to originate from epithelial progenitor cells of the choroid survival, resection alone is associated with poor outcomes
[84]
plexus . CPCs share a similar incidence rate as CPTs, due to the rapid progression of the disease . Radiotherapy
[71]
with approximately 20% occurring in the adult population is considered an option for extending survival but can
and 80% occurring in the pediatric population [2,72] . only be used in patients above the age of 3 who requires
Previous studies have found associations between CPCs narrow radiation fields. Consequently, radiation therapy
and Li-Fraumeni syndrome, which carries TP53 germline is an unlikely option in most cases, considering that CPCs
mutations [34,73] . In addition, other studies have identified typically occur at a young age and are often of significant

associations between CPCs and somatic TP53 mutations. size. However, patients below the age of 3 can receive
Furthermore, spontaneous TP53 germline mutations have adjuvant chemotherapy, which can contribute to long-term
been linked to cases of CPCs . survival but cannot prevent CPC recurrence [3,80,83,84] .
[74]
The WHO classifies CPCs as grade III CPTs with >5 CPCs carry a poor prognosis due to the challenges in
mitotic figures per 10 HPFs [27,64] CPCs exhibit characteristics achieving gross total resection, which is accomplished in
such as high mitotic activity, necrosis, high cellularity, only 40 – 50% of cases, because of the invasiveness of the
loss of papillary growth pattern, and invasion of brain mass and the high risk of intraoperative hemorrhage [85-87] .
parenchyma [75,76] . Grossly, CPCs are friable, soft, globular Patients with CPCs have a median survival of 2.5 – 3 years.
masses with irregular projections, high vascularity, and Moreover, worse outcomes have been observed in patients
variable adherence to ventricular walls, along with invasion identified with TP53 mutation using immunohistochemistry
into adjacent brain parenchyma. Sectioning revealed solid (Figure 1 and Table 1) [34,75,88] .
areas of intermixed necrotic and hemorrhagic foci [77-79] . On
immunohistochemistry, CPCs are positive for cytokeratin, 4. Emerging treatments and preclinical
GFAP, S-100, transthyretin, and vimentin. GFAP is interventions
positive in roughly 20% of CPC cases, and positive staining Emerging treatments for CPTs include advancements that
for S-100 and transthyretin is typically less frequent than supplement the conventional surgical intervention but may
in CPPs [2,76,80] . CPCs may also exhibit positive staining serve as standalone treatments in the future. Emerging
for p53, which is associated with Li-Fraumeni syndrome treatments for CPTs can be categorized as neuroendoscopy,
in families carrying TP53 germline mutations, somatic laser interstitial thermal therapy (LITT), photothermal
TP53 mutations, and spontaneous TP53 germline therapy (PTT), immunotherapy, manipulation of the
mutations [34,73,74,81] . CPCs are most commonly in the lateral tumor microenvironment (TME), gene therapy, epigenetic
ventricle (50%), fourth ventricle (40%), and third ventricle modulators, and nanotechnology.
(5%). Roughly 5% of cases involve multiple ventricles, and
rarely, CPCs can occur in the cerebellopontine angle, near 4.1. Neuroendoscopy
the foramina of Lushka, as a suprasellar mass, or as an The use of endoscopy in neurosurgery, known as
intraparenchymal mass [77-79] . neuroendoscopy, allows for maximal resection in a
The pathophysiology of the clinical symptoms observed minimally invasive manner without the need for brain
in CPCs is proposed to be similar to the previously discussed retraction by utilizing the natural cavity of the ventricular

Volume 2 Issue 2 (2023) 4 https://doi.org/10.36922/td.1057