Choroid plexus tumours are rare types of cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs that develop in the choroid plexus, a complex network of bloodthe red bodily fluid that transports oxygen and other nutrients around the body vessels and cellsthe basic structural and functional unit of all living things found in the lining of most of the ventricles in the brain. The main function of the choroid plexus is to produce and secrete cerebrospinal fluid (CSF), which provides protection, nourishment, and waste removal in the central nervous system (CNS). The CNS is responsible for all sensory and motor functions in the body, and is composed of the brain and spinal fluid.
Choroid plexus tumours are slightly more common in females, and are most commonly diagnosed in children under the age of two. However, anyone can develop this disease.
Types of Choroid Plexus Tumours
There are three primary types of choroid plexus tumours, which are classified by the type of cells they develop from.
Choroid Plexus Papilloma
Choroid plexus papillomas are the most common type of choroid plexus tumours, and are often benignnot cancerous, can grow but will not spread to other body parts. They are generally considered low gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells tumours (or grade I tumours), as they often develop slowly and rarely metastasise. Choroid plexus papillomas often have a very good prognosisto predict how a disease/condition may progress and what the outcome might be.
Atypical Plexus Papilloma
Atypical plexus papillomas, also known as atypical choroid plexus papillomas, are the least common type of choroid plexus tumoura tissue mass that forms from groups of unhealthy cells. They are generally considered intermediate grade tumours (or grade II tumours), as they often develop slowly and rarely metastasise, but can behave more aggressively than choroid plexus papillomas. Atypical plexus papillomas generally have a good prognosis, but are likely to recur after treatment.
Choroid Plexus Carcinomas
Choroid plexus carcinomas are a less common type of choroid plexus tumours, and are often malignantcancerous, may grow and spread to other areas of the body (cancerous). They are generally considered high grade tumours (or grade III tumours), as they are often aggressive and likely to metastasise. Choroid plexus carcinomas may not have as good of a prognosis as other types of choroid plexus tumours.
Treatment
When cancers are detected, they are staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancer cells look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas and grading helps your doctors determine the best treatment for you. However, brain cancers are rarely staged, as they rarely spread to other parts of the body. Instead, they are generally graded from grades I-III.
Choroid plexus tumours are generally graded by subtype:
- Grade 1 (low grade) tumours: cancer cells present as slightly abnormal and are usually slow growing. Includes choroid plexus papillomas.
- Grade II (intermediate grade) tumours: cancer cells present as abnormal and grow faster than grade-I tumours. Includes atypical plexus papillomas.
- Grade III (high grade) tumours: cancer cells present as very abnormal and grow quickly. Includes choroid plexus carcinomas.
Once your tumour has been graded, your doctor may recommend genetic testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment is dependent on several factors, including location, age, stage of disease and overall health.
Treatment options for choroid plexus tumours may include:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence to remove as much of the tumour as possible.
- Shunt insertiona procedure to relieve build-up of cerebrospinal fluid (CSF) in the brain.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Immunotherapya treatment that uses a person's immune system to fight cancer.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Some of the information regarding treatment was obtained from the Choroid Plexus Tumour: Diagnosis and Treatment page published by the National Cancer Institute.
Risk factors
Because of how rare choroid plexus tumours are, there has been limited research into the riskthe possibility that something bad will happen factors of this disease. However, certain genetic mutations and Li-Fraumeni syndrome have been linked to this disease.
Symptoms
The main symptoms of choroid plexus tumours result from hydrocephalusa build up of cerebrospinal fluid (CSF) in the brain, causing pressure and potentially damaging brain tissue, and may cause:
- Nauseato feel sick or likely to vomit and/or vomiting (generally worse in the morning).
- Irritability.
- Headaches (often after waking up in the morning).
- Blurred or double vision.
- Seizures.
- Lethargylack of energy, fatigue.
- Fatiguea state of extreme tiredness or exhaustion, can be physical or mental.
- Problems feeding and/or walking.
- Enlarged fontanelles (soft spots on an infant’s head between the bony plates of the skull).
- Enlarged head (more common in infants).
Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.
Diagnosis
If your doctor suspects you have a choroid plexus tumour, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Neurological examinationan assessment of sensory and motor functions, such as vision, balance and coordination.
- Imagingtests that create detailed images of areas inside the body tests, most commonly a MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- Lumbar puncturea procedure that involves inserting a needle between two vertebrae in the lower spine and extracting a sample of cerebrospinal fluid (CSF) for analysis.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
