A choroid plexus carcinoma is a malignancy in the parts of the brain that produce and contain cerebrospinal fluid (CSF). These areas, called ventricles, are lined with specialized cells that produce the fluid. When they become malignant, cancer can spread through the ventricles and intrude on the brain. This cancer is most commonly seen in children, and it is very rare. Only a small fraction of pediatric brain tumors take the form of choroid plexus carcinoma.
Cases of this cancer are usually diagnosed around the first year of life. Symptoms can include difficulty feeding, bulges in the skull, and difficulty tracking objects or acquiring motor skills. Since very young children may not be able to report symptoms like headaches and double vision, it is important to pay attention to signs of neurological impairment in young children. A neurologist can evaluate a child to determine if delays in development or peculiar behaviors are a cause for concern.
The five year survival rate for patients with choroid plexus carcinoma is about 40%. Some things that can influence the survival rate include the time of diagnosis, the surgeon’s level of skill, and the kind of treatment received. Diagnosis can include medical imaging studies to look at the brain, as well as evaluation of brain tissue after surgery. In surgery, it is critical to completely remove the tumor so it cannot grow back. If malignant tissue remains, the cancer can recur.
Patients with this condition may benefit from chemotherapy treatments to slow or stop the growth of cancer cells. A doctor can work with patients and family to develop an effective and appropriate treatment plan, after considering all the factors in the case. Sometimes treatment may need to be adjusted, as may be the case after surgery if the care team discovers the choroid plexus carcinoma is more involved than it appeared to be on scans. The patient’s response to chemotherapy and other treatments is also a consideration.
It is important to avoid confusing this cancer with choroid plexus papilloma (CPP). The two neoplasms are very different; choroid plexus papilloma is benign, and tends to respond very well to treatment. Patients diagnosed with this condition still need treatment and monitoring to check for signs of recurrence, but they are in less danger than choroid plexus carcinoma patients. In both cases, the doctor should be able to provide information about treatment options, outcomes with different options, and long term survival for the patient.
