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Choroid Plexus Carcinoma

Choroid plexus carcinoma is a cancerous type of tumour that develops in the ventricles of the brain. These structures contain cerebrospinal fluid (CSF), the fluid that provides protection for the spinal cord and the brain. Cells of the choroid plexus produce CSF.

As choroid plexus carcinoma gets larger, it can block the flow of CSF or cause an excess of CSF. These conditions can lead to increased pressure on the brain and related symptoms such as nausea, vomiting, and headaches.

Choroid plexus carcinoma is rare and occurs most commonly in infants. Cancerous tumours in the choroid plexus are much less common than noncancerous tumours in the same region.

Symptoms

Common symptoms of a choroid plexus carcinoma include:

  • Headache
  • Nausea
  • Vomiting
  • Irritability
  • Lack of energy

It’s important to remember that infants and younger children with a choroid plexus carcinoma will not be able to vocalise their symptoms in the same way older children and adults do. Infants and toddlers may struggle to feed or walk, while parts of the skull that have not completely hardened may feel full, as if there is a slight bulge.

Causes & Risk Factors

The cause of choroid plexus carcinoma, specifically, is not known. Like with any cancerous mass, a choroid plexus carcinoma forms when genetic mutations occur that alter the body’s normal processes for stopping tumours from forming. Choroid plexus carcinoma has been linked to one inherited syndrome, known as Li-Fraumeni syndrome, in very rare cases.

The only known risk factor for developing choroid plexus carcinoma is having a parent with Li-Fraumeni syndrome. Li-Fraumeni syndrome is a rare genetic disorder that drastically increases your risk of developing cancer.

Diagnosis & Complications

A doctor will use the medical history and current symptoms of a patient with suspected choroid plexus carcinoma to make a diagnosis, along with a variety of neurological tests.

Some diagnostic tests that may be used include:

  • Neurological exam – this tests the functioning of the 5 senses, as well as balance, coordination, and reflexes.
  • Imaging tests – a computerised tomography (CT) scan or magnetic resonance imaging (MRI) scan of the brain will show a tumour.
  • Genetic tests – if the mutation causing the choroid plexus carcinoma is suspected to be passed down from a parent, genetic tests may be performed on both the parent and the child.

The main complication of choroid plexus carcinoma is the possibility of brain swelling, which can be fatal if not treated quickly.

Treatment & Management

It’s important to keep in mind that treatment of choroid plexus carcinoma is different for children than for adults, and a specialist will treat children diagnosed with choroid plexus carcinoma.

Treatment options include:

  • Surgery – this can be used to remove as much of the cancer as possible. Sometimes, the entire tumour can be removed; other times, that’s not possible. However, surgery can be a good option for aggressively treating choroid plexus carcinoma. It can also be used to relieve fluid that is causing swelling in the brain.
  • Chemotherapy – this treatment uses medicine to kill the cancer. It is often used with surgery or radiation therapy.
  • Radiation therapy – this treatment uses X-rays, protons, and other energy sources to kill cancer. Radiation is a good option for preserving healthy tissue while killing cancer. It can also be used with chemotherapy or surgery to keep cancer from coming back.

Patients with choroid plexus carcinoma and their families can discuss treatment options that are right for them with their doctor.

 
 
 

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