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Tumors may arise in the choroid plexus, tissue located in the spaces of the brain called the ventricles. The choroid plexus makes the cerebrospinal fluid (CSF), which surrounds the brain and spinal cord. Choroid plexus tumors are rare, representing only 3 percent of pediatric brain tumors, but account for 10-20 percent of pediatric brain tumors that occur within the first year of life. The malignant form, choroid plexus carcinoma, accounts for 10-20 percent of all choroid plexus tumors. Males and females are equally affected by this type of tumor.
As you read further below, you will find general information about choroid plexus tumors. If you would like to view summary information about brain tumors first, see the overview on brain tumors.
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Symptoms vary depending on size and location of tumor. This tumor can block the normal flow of CSF, causing increased pressure on the brain (hydrocephalus) and enlargement of the skull and a variety of symptoms. Common symptoms may include:
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- headaches ( especially upon awakening)
- nausea and vomiting (especially upon awakening)
- lethargy and irritability
- problems feeding or walking
- enlarged head size or fontanels (the soft "spot" that occurs before the bones in the head become solid) in infants
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The symptoms of a brain tumor may resemble other conditions or medical problems - ranging from the simple to the serious. Always consult your child's physician for diagnosis and treatment.
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These tumors occur in two different types: papillomas and carcinomas. An intermediate form is rarely seen.
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- choroid plexus papilloma (CPP) is a slow-growing tumor (referred to as "benign") and only rarely spreads to other parts of the brain and spinal cord.
- choroid plexus carcinoma (CPC) is a malignant, aggressively growing cancer and is more likely to spread (metastasize).
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Diagnostic procedures for a choroid plexus tumor, like other brain tumors, are necessary to determine the exact type of tumor and whether the tumor has spread. These may include:
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- physical exam - your child's physicians can test neurologic function including: reflexes, muscle strength, eye and mouth movement, coordination, and alertness.
- scans - magnetic resonance imaging (MRI) scans are obtained using computerized large magnetic fields to produce detailed images of organs and structures within the body. This will provide information on the location, size and characteristics of the tumor. The magnetic fields are not harmful and are safer than even routine x-rays. Computerized tomography (CT) or CAT scans using x-rays are also occasionally used.
- biopsy - a tissue sample from the tumor taken during surgery to remove part or all of the tumor. The sample will provide definitive information about the type of tumor.
- lumbar puncture (spinal tap) - to remove a small sample of cerebrospinal fluid (CSF), a special needle is placed into the lower back, into the spinal canal, the area around the spinal cord. CSF is the fluid that bathes the brain and spinal cord. The sample is sent for testing to determine if any tumor cells have started to spread. In children, this procedure is safely performed under sedation, and is less difficult and less painful than placing an intravenous (IV) catheter.
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Specific treatments to cure choroid plexus tumors will be determined by your child's physician based on:
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- type, location, and size of the tumor
- extent of the disease
- your child's age, overall health, and medical history
- how your child's doctors expects the disease to progress
- your child's tolerance for specific medications, procedures, or therapies
- your preference and judgement
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Treatment may include (alone or in combination):
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- surgery - usually the first step and mainstay in the treatment of brain tumors. The goal is to remove as much of the tumor as possible while preserving neurological function. Tumor specimens are examined by neuropathologists to determine the exact diagnosis. Complete resection or surgical removal of the entire tumor is ideal when this is feasible. However, tumor location and other characteristics may limit removal to a partial or sub-total resection. A biopsy is the surgical removal of a sample of the tumor for diagnostic purposes. This is frequently done if the tumor is in an area with sensitive structures around it that may be injured during removal. In general, the more complete the resection, the better the chances for cure.
- ventriculo-peritoneal shunt (VP shunt) - when a tumor causes blockage of CSF flow leading to symptoms, special tubing can be surgically implanted in the ventricles to drain excess CSF into the abdomen. This bypasses the tumor blockage and relieves symptoms of hydrocephalus.
- radiation therapy - precisely targeted and dosed radiation is used to kill cancer cells left behind after surgery. This therapy is important to control the local growth of tumor. If the tumor has spread, radiation to the whole brain and spinal cord are important to treat a choroid plexus tumor.
- chemotherapy - a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. For choroid plexus tumors, chemotherapy before surgery may help shrink the tumor, making it possible to remove.
Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors. Chemotherapy may be used alone for some types of cancer or in conjunction with other therapy such as radiation or surgery. Often, a combination of chemotherapy drugs is used to fight a specific cancer. Certain chemotherapy drugs may be given in a specific order, depending on the type of cancer it is being used to treat. While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.
Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:
- as an injection into the muscle or fat tissue
- intravenously (directly to the bloodstream; also called IV)
- intrathecally - chemotherapy given directly into the spinal column with a needle
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Total surgical removal of the tumor is usually all the treatment needed for 75-100 percent of patients with choroid plexus papilloma (CPP), although may will require a shunt (see below). For patients with choroid plexus carcinoma, surgical removal of the tumor is important, but additional treatment, usually radiation and chemotherapy, is needed (see below)
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- follow-up care - to manage disease complications, to screen for early recurrence of tumor and to manage late effects of treatment, regular visits with physical exams, laboratory testing, and scans are routine follow-up. In addition to neurosurgeons, radiation oncologists, pediatric oncologists and neurologists, the endocrinologists, neuro-psychologists, alternative/complementary therapy specialists, school liaison and psychosocial personnel continue to serve as members of the neuro-oncology team.
- rehabilitation - speech, physical, and occupational therapists are also part of the neuro-oncology team, and may be involved during and after treatments.
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Choroid plexus papillomas are usually cured by surgical removal of the tumor. Choroid plexus carcinomas are more malignant. Surgical removal is important, but often not curative without the addition of other types of treatment.
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Children's Hospital and Dana-Farber Cancer Institute are conducting numerous research studies that will help clinicians better understand and treat choroid plexus tumors. For more information on current research, see the Brain Tumor Program.
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Choroid Plexus Tumor
