Choroid plexus carcinoma (CPC) is a rare and aggressive type of brain tumor. This cancer originates in the choroid plexus, specialized tissue within the brain responsible for producing cerebrospinal fluid. While it can occur at any age, CPC primarily affects young children and infants.
Understanding Choroid Plexus Carcinoma
The choroid plexus is a network of cells and capillaries located within the ventricles, which are fluid-filled spaces deep inside the brain. Its primary function involves producing cerebrospinal fluid (CSF), a clear fluid that cushions the brain and spinal cord, transports nutrients, and removes waste products. This fluid also plays a role in maintaining proper brain pressure.
Choroid plexus carcinoma is a malignant tumor that develops from these CSF-producing cells. The World Health Organization (WHO) classifies it as a Grade III tumor, indicating its fast-growing and aggressive nature. It is a very rare tumor, accounting for less than 1% of all brain tumors.
Recognizing the Signs
The symptoms of choroid plexus carcinoma often arise from increased pressure within the skull, known as intracranial pressure. This pressure can result from the tumor itself growing, overproducing CSF, or obstructing the normal flow of CSF. These symptoms can be non-specific, making early diagnosis challenging.
In infants, signs may include a rapidly increasing head circumference or a bulging soft spot (fontanelle). Older children might experience persistent headaches, often worse in the morning, nausea, vomiting, or vision problems like blurred or double vision. Lethargy, irritability, developmental regression, and seizures are also common.
Diagnosis and Treatment Approaches
Diagnosing choroid plexus carcinoma typically begins with a neurological examination to assess a child’s reflexes, coordination, and overall neurological function. Imaging studies play a central role in identifying the tumor and determining its characteristics. Magnetic Resonance Imaging (MRI) of the brain and spine is the preferred method, providing detailed images of the tumor’s size, location, and any potential spread within the central nervous system. Computed Tomography (CT) scans may also be used as an initial imaging tool, particularly for very young children.
A definitive diagnosis relies on a tissue biopsy, which is usually obtained during surgery. This tissue is then examined by a pathologist to confirm the presence of cancer cells and classify the tumor. In some cases, cerebrospinal fluid analysis may also be performed to check for the presence of tumor cells if spread is suspected.
Treatment for choroid plexus carcinoma is complex and often involves a multidisciplinary team of specialists. Surgical removal of the tumor is the cornerstone of treatment, with the goal of achieving maximal safe resection.
Following surgery, chemotherapy is frequently used, particularly for any remaining tumor cells or in younger patients where radiation therapy may be delayed or avoided. Radiation therapy may be considered for older children, in cases where complete surgical removal was not possible, or for recurrent disease. Its use in very young children is carefully considered due to potential long-term side effects on brain development.
Outlook and Support
The outlook for individuals with choroid plexus carcinoma has improved with advancements in treatment, although it remains an aggressive cancer. Factors influencing the prognosis include the extent of surgical removal, the child’s age at diagnosis, and whether the cancer has spread.
Long-term follow-up care is essential, involving regular imaging and monitoring for any signs of recurrence or treatment-related side effects. Patients may also require ongoing supportive care, including rehabilitation services, to address any neurological or developmental impacts. Families coping with a diagnosis of a rare pediatric cancer like CPC can benefit from psychosocial support and connecting with patient advocacy organizations and support groups.