choroid plexus tumors

Choroid Plexus Tumors

Choroid plexus tumors are rare tumors that primarily occur in children (two years or younger).

These tumors arise from the choroid plexus, the cells that line the fluid-filled cavities (called ventricles) of the brain. The choroid plexus is responsible for producing cerebrospinal fluid (CSF), which is a clear fluid that surrounds the brain and acts as a watery cushion to protect it within the skull.

Choroid plexus tumors are divided into two types — papillomas and carcinomas. Choroid plexus papilloma is a benign (noncancerous) lesion that does not spread. Choroid plexus carcinoma is a malignant (cancerous) lesion that is able to spread to other parts of the brain via the cerebrospinal fluid.

 

What are the symptoms of choroid plexus tumors?

Because choroid plexus tumors grow within the fluid-filled ventricles of the brain, they often block the flow of cerebrospinal fluid. This can cause a buildup of cerebrospinal fluid that increases pressure in the brain, a condition called hydrocephalus. Some common symptoms, resulting from increased pressure in the brain, may include the following:

  • Headaches (typically in the morning)
  • Nausea or vomiting (typically worse in the morning)
  • Fatigue
  • Irritability
  • Enlarged head, including the soft spot (called the fontanel) in an infant’s head before the bones fuse together
How common are choroid plexus tumors?

Together, choroid plexus papilloma and carcinoma account for a minority of childhood brain tumors. In 2017, an estimated 150 new cases were diagnosed in the United States.1 The vast majority of choroid plexus tumors are the benign choroid plexus papillomas.2

Choroid plexus carcinoma, although relatively rare, is more common in patients with Li-Fraumani syndrome, a rare genetic disorder that increases risk for a variety of cancers.3 Children with choroid plexus carcinoma may be screened to help guide future treatment. Parents of children with choroid plexus carcinoma may also benefit from genetic counseling.

What are the treatment options for choroid plexus tumors?

Choroid plexus tumors are most effectively treated with surgery, to remove as much of the tumor as possible. With choroid plexus papillomas, surgery is often enough to be curative. Often, a procedure called embolization is performed by interventional neuroradiologists to block a blood vessel leading to the tumor. If this is possible, it reduces the risks associated with surgery. Patients with choroid plexus carcinomas also have improved outcomes when the tumor is completely removed, but are more successfully treated with a combination of therapies.

Aggressive chemotherapy, which may be provided as high-dose chemotherapy with stem cell transplant, is often recommended for patients with choroid plexus carcinomas. Several preliminary studies indicate that this improves patient outcomes, especially in those without complete surgical removal of the tumor.2 

Radiation therapy may also be used in combination with surgery and chemotherapy. While it can be an effective treatment for choroid plexus carcinomas, high doses can be toxic to the developing brain of young children. Radiation therapy is usually reserved for patients whose tumors have recurred after initial treatment, or for those who have metastatic disease and do not respond to chemotherapy.

What are the outcomes for patients with choroid plexus tumors?

A choroid plexus tumor is a serious condition that needs to be treated by a multidisciplinary team consisting of neurosurgeons, neuro-oncologists and radiation oncologists. However, many patients have positive long-term outcomes, especially those with choroid plexus papilloma and complete surgical removal of the tumor.

For patients with choroid plexus carcinoma, long-term outcomes depend on a variety of factors, including the size and location of the tumor, whether it spread (or metastasized) to other brain regions, and whether it was completely removed during surgery. In general, complete surgical removal of the tumor offers the best patient outcomes.

However, patients with choroid plexus carcinoma often require more aggressive treatments, such as chemotherapy and/or radiation after surgery. Although these treatments can be effective for many patients, some survivors are at risk for developing long-term side effects. Especially for pediatric patients, radiation and chemotherapy may increase risk of learning disabilities, hearing loss, fertility issues, or secondary cancers (like leukemia) that can develop later in life. However, your doctors will discuss how these risks apply to your or your child’s personalized treatment plan. Ongoing research at UCSF aims to identify more effective treatment strategies with fewer long-term complications to the patient.

References
  1. Ostrom, Q.T., et al., CBTRUS Statistical Report: Primary brain and other central nervous system tumors diagnosed in the United States in 2010-2014. Neuro Oncol, 2017. 19(suppl_5): p. v1-v88.
  2. Sun, M.Z., et al., Current management of choroid plexus carcinomas. Neurosurg Rev, 2014. 37(2): p. 179-92; discussion 192.
  3. Malkin, D., Li-fraumeni syndrome. Genes Cancer, 2011. 2(4): p. 475-84.

This content was reviewed by UCSF pediatric neurosurgeon Nalin Gupta MD, PhD.