Choroid plexus tumor

We understand that you may have a lot of questions when your child is diagnosed with a choroid plexus tumor:

• What exactly is it?
• What are potential complications in my child’s case?
• What are the treatments?
• What are possible side effects from treatment?
• How will it affect my child long term?

We’ve tried to provide some answers to those questions here, and when you meet with our experts, we can explain your child’s condition and treatment options fully.

How are choroid plexus tumors classified?

An important part of diagnosing a brain tumor involves staging and classifying the disease, which will help your child’s doctor determine treatment options and prognosis. Staging is the process of determining whether the tumor has spread and if so, how far.

There are three main types of choroid plexus tumors: papillomas, atypical papillomas and carcinomas:

Choroid plexus papillomas (CPP) and atypical papillomas (APP):

• grow more slowly ("benign")
   
• are by far more common, accounting for 80 to 90 percent of choroid plexus tumors
   
• only rarely spread to other parts of the brain and spinal cord

Choroid plexus carcinomas (CPC):

• grow more aggressively (“malignant”)
   
• are more likely to spread (metastasize)
   
• are less common, accounting for 10 to 20 percent of all choroid plexus tumors.

In rare cases, a choroid plexus tumor may appear to have characteristics of both CPP and CPC.

Can a choroid plexus papilloma turn into a choroid plexus carcinoma?

Generally speaking, these are two different kinds of tumors, and usually a CPP stays a CPP. Tumors have a wide spectrum in terms of how harmful they are, and sometimes a CPP may develop some unusual features, but usually not enough to fulfill the criteria for a CPC. APP may occasionally behave like a CPP.

Causes

What causes choroid plexus tumors?

It’s important to understand that these tumors most often have no known cause.There’s nothing that you could have done or avoided doing that would have prevented them from developing.There is one rare genetic disease called Li-Fraumeni Syndrome that can be associated with CPP.  If your child has a CPP, his doctors will talk to you about genetic testing for this disease.

Symptoms

What are the symptoms of a choroid plexus tumor?

Symptoms vary depending on size and location of tumor. A choroid plexus tumor can block the normal flow of cerebrospinal fluid, causing increased pressure on the brain (hydrocephalus) and enlargement of the skull. It can also cause symptoms including:

• headaches (generally upon awakening in the morning)
   
• nausea and vomiting (often worse in the morning and improving throughout the day)
   
• lethargy and irritability
   
• problems feeding or walking
   
• enlarged fontanels (the soft "spot" that occurs before the bones in the head become solid) in infants

Keep in mind that these symptoms may resemble other, more common conditions or medical problems. If you don’t have a diagnosis and are concerned, always consult your child's physician.

Questions to ask your child’s doctor

After your child is diagnosed with a brain tumor, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you.

Lots of parents find it helpful to jot down questions as they arise – that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.

If your child is old enough, you may want to suggest that she write down what she wants to ask her health care provider, too.

Some of the questions you may want to ask include:

• Has my child’s brain tumor spread?
   
• Can the tumor be treated with surgery?
   
• How long will my child need to be in the hospital?
   
• What are the possible short and long-term complications of treatment? How will they be addressed?
   
• What is the likelihood of cure?
   
• What services are available to help my child and my family cope?

FAQ

Q: Will my child be OK?
A: A child’s long-term health after treatment for a choroid plexus tumor varies significantly depending on whether the tumor has spread and whether it can be completely removed through surgery. Choroid plexus papillomas are generally more likely to be successfully treated than choroid plexus carcinomas. Your doctor will discuss treatment options with you and your family including clinical trials and supportive care.

Q: How are choroid plexus tumors treated?
A: Choroid plexus papillomas and atypical choroid plexus papillomas are usually treated by surgical removal of the tumor. Choroid plexus carcinomas are often harder to treat. It’s important to remove the tumor, but treatment also requires additional therapies such as chemotherapy and/or radiation.

Q: What services are available to help my child and my family cope?
A: We offer a variety of services to help you, your child and your family get through this difficult time. Read more about our support services.

Q: Where will my child be treated?
A: Children treated on an outpatient basis through Dana-Farber/Children’s Hospital Cancer Center are cared for at the Jimmy Fund Clinic on the third floor of the Dana-Farber Cancer Institute. If your child needs to be admitted to the hospital, she will stay at Children’s Hospital Boston on the ninth floor of the Berthiaume Building.

Q: What kind of supportive or palliative care is available for my child?
A:When appropriate, our Pediatric Advanced Care Team (PACT) offers supportive treatments intended to optimize the quality of life and promote healing and comfort for children with life-threatening illness. PACT can also provide psychosocial support and help arrange end-of-life care when necessary.