Pilocytic Astrocytoma Causes

In most cases, the cause of pilocytic astrocytoma is unknown. The majority of cases develop spontaneously. Children with Neurofibromatosis Type 1 (NF-1), an inherited genetic syndrome, are at increased risk of developing these tumors, specifically in the optic pathway or hypothalamus. Other genetic conditions that have been shown to put patients at higher risk for pilocytic astrocytomas are Li-Fraumeni syndrome, tuberous sclerosis complex, nevoid basal cell carcinoma syndrome, and Turcot syndrome. An exposure to radiation therapy for treatment of other cancers is also a risk factor.

Pilocytic Astrocytoma Symptoms

The symptoms of pilocytic astrocytomas depend on the area of the brain the tumor is in. If the tumor is mostly in the cerebellum, you may see changes in the way your child walks. They may start to fall or have issues with coordination. Tasks they were able to complete easily before are now more difficult. Other symptoms caused by cerebellar pilocytic astrocytomas are signs of increased intracranial pressure. This is a result of the tumor blocking the flow of cerebral spinal fluid (CSF) out of the brain. This is called obstructive hydrocephalus. Signs and symptoms of this include headache, vomiting often in the morning, poor balance and fatigue.

If the pilocytic astrocytoma involves the optic pathway, signs and symptoms may include blurred vision, double vision or rapid eye movements.

Hypothalamic JPAs can present with similar symptoms as those involving the optic pathway. Parents may also see their child not feeding well, weight loss, or other endocrinology deficits. In extreme cases, JPAs in the hypothalamic area can cause children to present with diencephalic syndrome. This syndrome occurs in infants and young children. It is characterized by failure to thrive, progressive thinness and weakness, and hyperkinesia (hyperactivity and restlessness).

Pilocytic Astrocytoma Diagnosis

The best way to see these types of tumors in the brain is by magnetic resonance imaging (MRI). Sometimes, pilocytic astrocytomas are seen on computed tomography (CT) scans first, as they can be done quicker. Then an MRI is performed at a later date. Pilocytic astrocytomas have a very distinct presentation on imaging.

To confirm this diagnosis, a piece, or all of the tumor, will be removed by a neurosurgeon. The tumor tissue will then be evaluated by a pathologist.

Pilocytic Astrocytoma Treatment

The most common treatment for pilocytic astrocytoma is surgery to remove as much of the tumor as possible. In some cases, chemotherapy and radiation is required as well to treat this tumor. This decision will be made by your oncologist based on your child’s specific situation and the tumor’s location.

Pilocytic Astrocytoma Prognosis

The prognosis of pilocytic astrocytoma depends on the amount of tumor that is surgically removed. The survival rate at 10 years is approximately 95% if the tumor is completely removed.