Choroid Plexus Tumors - Causes, Symptoms, Diagnosis, and Treatment

What Are Choroid Plexus Tumors?

Choroid plexus tumors are uncommon primary brain tumors developed from differentiated epithelial tissue. With an annual frequency of 0.3 occurrences per million, these tumors account for 0.4 to 0.8 percent of all brain tumors. The ventricles are the most often reported places. Tumors are designated choroid plexus-carcinoma (WHO grade III) when they exhibit nuclear pleomorphism and necrosis. Furthermore, these tumors express carcinoembryonic antigen (CEA) in peripheral blood.

How Are Choroid Plexus Tumors Classified?

The primary central nervous system (CNS) is graded depending on the location, kind, extent of tumor genetic findings, dissemination, the amount of tumor that remains after surgery, and the patient's age.

Choroid plexus tumors are classified into three classes depending on their features.

Grade I - Choroid plexus papillomas are benign tumors of the choroid plexus. This indicates that the tumor cells develop slowly.

Grade II - Atypical choroid plexus papillomas are mid-grade tumors. This suggests that the tumors have a higher likelihood of returning following removal.

Grade III - Choroid plexus carcinomas are cancerous. This means they are rapidly developing tumors that tend to infect adjacent tissue.

What Is the Etiology of Choroid Plexus Tumors?

Cancer is a genetic illness caused by gene alterations that cause cancer cells to develop and spread. The etiology of the majority of choroid plexus tumors is unknown. However, certain choroid plexus malignancies have been connected to genetic alterations. In addition, specific gene alterations handed down through families have been related to an increased risk of developing choroid plexus carcinomas on rare occasions.

Some choroid plexus tumors have been associated with SV40 infections, although they might also be impacted by other variables, such as X-chromosome-linked disorders. Cerebellopontine angle tumors were linked to being older, having benign histology, and being female. Conditions with X-chromosome abnormalities are related to choroid plexus papilloma. SV40 is more commonly identified in child tumors than in adult choroid plexus tumors.

Where Does the Choroid Plexus Originate From?

• Choroid plexus cancers develop from the choroid plexus, a region in the brain.

• It lines the brain's ventricles and the fluid-filled chambers of the brain and is responsible for cerebrospinal fluid (CSF) production.

• Choroid plexus cancers nearly always arise from the ventricles; however, they can also develop in other parts of the central nervous system.

What Are the Clinical Signs and Symptoms of Choroid Plexus Tumors?

The signs and symptoms of choroid plexus tumors are as follows:

• Hydrocephalus (an abnormal increase of cerebrospinal fluid (CSF) in the brain).

• Seizures.

• Nausea.

• Irritability.

• Double vision or blurred vision

• Vomiting.

• An intense urge to sleep.

• Headaches.

What Is the Occurrence of Choroid Plexus Tumors in Infants?

Choroid plexus tumors represent fewer than one percent of juvenile brain tumors and are often diagnosed in newborns under three. Some are linked to cancer-predisposing disorders, such as the Aicardi and LiFraumeni syndromes. Aicardi syndrome manifests either in utero or at birth. The Li-Fraumeni syndrome is associated with a high prevalence of TP53 germline mutations. Like other members of the choroid plexus tumors group, hydrocephalus is the most common initial finding, followed by macrocephaly and an intracranial tumor discovered by imaging. Choroid plexus tumors are invasive and typically metastasize when they are diagnosed.

How Are Choroid Plexus Tumors Diagnosed?

Magnetic Resonance Imaging (MRI) -

• Choroid plexus tumors are most commonly found in the brain's ventricles.

• Uneven boundaries and a cauliflower-like look characterize them.

• Tumors frequently augment with contrast, and many tumors can simultaneously be observed in various central nervous system regions.

• Choroid plexus carcinomas may have edema surrounding them, which can be noticed on magnetic resonance imaging (MRI).

Immunohistochemical Findings -

• Choroid plexus tumors are immunoreactive with vimentin and cytokeratin but not with transthyretin or s-100 protein.

• An enhanced mitotic rate is utilized to determine if a choroid plexus tumor is malignant or not.

• Choroid plexus tumors have a Ki67/MIB1 labeling index of 1.9 percent.

• However, it should be noted that more excellent mitotic activity is only predictive in older children.

• Choroid plexus tumors have numerous histologic variations.

• Choroid plexus tumors vs. villous hypertrophy VH is described as a diffuse bilateral expansion of the ventricular choroid plexus.

• This variation has been identified as a significant cause of shunt-resistant hydrocephalus in fetuses and newborns due to increased cerebrospinal fluid production.

What Is the Treatment of Choroid Plexus Tumors?

• Brain tumor surgery is the primary treatment for all choroid plexus tumors, and it can result in a cure if complete resection is done.

• Tumor resection can be accomplished in various methods, with a strategy most suited to each patient's tumor.

• CA craniotomy is an open operation in which a portion of skull bone is temporarily removed to gain access to the brain.

• If hydrocephalus does not resolve following tumor removal, a shunt can be placed to redirect the cerebrospinal fluid (CSF).

• Choroid plexus carcinomas may not be removed entirely due to their propensity to invade adjacent tissue. In such cases, a subtotal resection is done to avoid injuring critical, healthy tissues.

• In order to treat the remaining tumor and prevent tumor recurrence, adjuvant treatment with radiation, chemotherapy, or both may be required.

• If the tumor recurs, excision, radiation, chemotherapy, or a combination of these treatments may be advised.

• Stereotactic radiosurgery may be performed as a noninvasive procedure in which substantial doses of radiation are targeted on tumor cells while leaving neighboring brain tissue unharmed.

• The regimen for young children is slightly different; radiotherapy is avoided in children under three due to probable detrimental effects on the growing brain; chemotherapy can be used instead.

Conclusion

Choroid plexus tumors develop from the choroid plexus, a tissue in the brain. It lines the ventricles (fluid-filled chambers) of the brain and is responsible for the production of cerebrospinal fluid (CSF). It is an uncommon tumor that develops in the network of blood arteries and cells in the brain's fluid-filled regions. These tumors are more frequent in children under the age of two. Tumors of the choroid plexus can be benign (not cancerous) or malignant (cancerous). Craniotomy, endoscopic craniotomy, and shunt implantation are standard surgical therapies. However, they can form in any ventricles in adults and lateral ventricles in youngsters. Tumors can obstruct CSF circulation and induce CSF overproduction.