Choroid Plexus Tumors
What are choroid plexus tumors?
Choroid plexus tumors are a rare proliferation of cells arising from the choroid plexus, a vascular structure inside the ventricles of the brain. Choroid plexus tumors are divided into two types — papillomas and carcinomas. Together, these lesions constitute fewer than 1% of childhood brain tumors. Because these lesions grow within the ventricular system, they often obstruct the flow of cerebrospinal fluid, resulting in hydrocephalus.
Choroid plexus papillomas account for 60-70% of choroid plexus tumors and are benign lesions that do not spread within the ventricular system or brain. They usually present in the first year of life.
Choroid plexus carcinomas are malignant tumors that have the potential to spread throughout the ventricular system and brain. Choroid plexus carcinomas are more common in patients with Li-Fraumani Tumor predisposition syndrome. This syndrome results from inherited mutation of the TP53 gene, and children with choroid plexus carcinomas should be screened for this syndrome and parents may benefit from genetic counseling.
What is the outcome?
Patients with choroid plexus papillomas do extremely well with surgery alone. Pathologic diagnosis is essential to determine if additional therapy is required.
Patients with choroid plexus carcinomas have a more guarded prognosis and often require more aggressive treatments, such as chemotherapy and/or radiation after surgery.
How serious is it?
A choroid plexus tumor is a serious conditions that needs to be treated by a multi-disciplinary team consisting of neurosurgeons, neuro-oncologists and radiation oncologists. Even with appropriate therapies, a fraction of patients can die from this disease.
What are the treatment options?
As with most tumors, our treatments are limited to three modalities: surgery, radiation, and chemotherapy.
Surgery: An aggressive surgical resection is the most effective therapy for choroid plexus tumors. Surgery is often curative for choroid plexus papillomas, and patients with choroid plexus carcinomas have improved outcomes when the tumors can be completely removed.
Chemotherapy: Aggressive chemotherapy, with or without high dose chemotherapy with stem cell rescue, is often used in patients with choroid plexus carcinomas. Several small studies appear to show this improves outcome in patients, especially in patients with incomplete resections.
Radiation: Radiation can be an effective treatment for choroid plexus carcinomas; however, high doses can be toxic to the developing brain of young children. Therapy is often reserved for patients whose tumors have recurred after initial treatment, or for those with metastatic disease who do not respond to chemotherapy.
What clinical trials are testing new treatments for choroid plexus carcinomas?
Choroid plexus carcinomas are a rare tumor and there are no open clinical trials for newly diagnosed patients. If a child’s tumor recurs, or progresses through standard therapy, we offer a number of investigational trials through the Pediatric Brain Tumor Consortium and the Children’s Oncology Group.
Click here for a list of trials for pediatric brain tumors currently offered at UCSF. Or contact Dennis Aguilling at (415) 476-9387 for additional information.
What research is being done on choroid plexus carcinoma?
Research is focused on understanding the frequency and role of TP53 mutations in patients with choroid plexus carcinomas. An international registry has been established to follow these patients: http://www.choroidplexustumors.com/