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Choroid plexus tumors (CPTs) are rare tumors that begin in the ventricles of the brain. The ventricles are spaces in the brain that are filled with cerebrospinal fluid (CSF). Choroid plexus tumors may be benign or malignant. CPTs most often occur in infants, but they can develop at any age.
Choroid plexus tumors account for 3% of childhood brain tumors, but they represent 10-20% of brain tumors in infants under 1 year of age. Approximately 75-80 new cases of CPT are diagnosed annually in the United States in children and young adults under 21 years of age.
There are 2 main types of choroid plexus tumor:
The main treatment for choroid plexus tumor is surgery. Additional treatments including chemotherapy and radiation therapy may be used depending on the type of tumor and the extent of disease after surgery.
Symptoms of CPT vary based on the child’s age and tumor location. As the tumor grows, it blocks the normal flow of cerebrospinal fluid. This causes a build up of fluid within the brain known as hyrodcephalus. The fluid causes the ventricles to widen and increases pressure on the brain (intracranial pressure). Many of the symptoms of choroid plexus tumor are due to the increased pressure against brain tissue.
Choroid plexus tumor symptoms may include:
Doctors test for choroid plexus tumors in several ways. These tests include:
Depending on the histology and type of tumor, the choroid plexus tumor may be benign or malignant. CPTs are diagnosed as choroid plexus papilloma (CPP), noncancerous, or choroid plexus carcinoma (CPC), cancerous.
Tumors can be grouped by how they look under the microscope. The more abnormal cells look, the higher the grade. Choroid plexus papillomas are usually Grade I tumors and are generally benign. Choroid plexus carcinomas are typically grade III tumors. They are more aggressive in nature and grow very rapidly.
Staging of choroid plexus tumor involves obtaining an MRI of the spine and brain to look for the extent of spread of disease and a lumbar puncture to look for cancer cells in the CSF.
Chance of cure is very good if surgery can completely remove the tumor. With successful surgery for choroid plexus papilloma (CPP), survival rates approach 100%. Choroid plexus carcinoma (CPC) is more aggressive, but chance of cure is approximately 50-70%.
Factors that influence prognosis include:
Treatment depends on several factors including the size and location of the tumor, the child’s age, and the tumor types (CPP vs CPC). Treatment for choroid plexus tumors may include surgery, chemotherapy, and radiation. Choroid plexus papillomas and atypical choroid plexuses tumors are primarily managed by surgery when possible. Choroid plexus carcinoma is a more aggressive cancer, and most patients will receive multiple types of treatments.
Surgery is used to both diagnose and treat choroid plexus tumors. The goal of surgery is to remove as much of the tumor is possible. Rarely, surgery may not be possible due to the location of the tumor.
Children with choroid plexus papilloma often do not need any additional treatment if the tumor is completely removed. Children with choroid plexus carcinoma usually need chemotherapy and radiation in addition to surgery.
In some cases, a shunt is placed in the brain to keep fluid from building up in the brain (hydrocephalus). This fluid causes increased intracranial pressure and may cause many of the symptoms associated with choroid plexus tumors. A shunt is a small tube that drains cerebrospinal fluid to remove it from the brain, thereby lessening symptoms. The shunt may be temporary or permanent.
Chemotherapy is often used along with surgery and/or radiation to treat choroid plexus tumors, particularly CPC. Chemotherapy may systemic, regional or intrathecal.
Children with relapsed CPC who have not fully responded to chemotherapy and radiation may be offered treatment within a clinical trial. While there no biological agents specifically targeting the cancer cells in CPC available yet, patients may be eligible for onging immunotherapy trials and phase I studies.
Supportive care for patients with choroid plexus tumor includes appropriate rehabilitation and neurological consultation. Steroid and anti-seizure medications may be needed. Additional support may be needed to address issues in learning, developmental milestones, and coping with cancer.
Children who are long term survivors of CPT need close and prolonged monitoring for late effects of the treatment. Children with a germline TP53 mutation or Li-Fraumeni syndrome need referral to genetic counseling and surveillance for other cancers associated with this syndrome.
Reviewed: June 2018