What is a Cerebellar Juvenile Pilocytic Astrocytoma (JPA)?
Cerebellar Juvenile Pilocytic Astrocytoma (JPA) makes up about 15-25 percent of brain tumors seen in children. This type of tumor arises from an astrocyte, which is a type of glial cell. Astrocytes make up the supportive tissue of the brain. Astrocytes are named for their star-like appearance. Cerebellar astrocytomas arise from the cerebellum, which is the area of the brain responsible for controlling coordinated movement, balance and posture. Cerebellar JPA tend to occur before the age of 10 years, most commonly between the ages of 6 and 9 years of age. Patients with cerebellar JPA have an excellent prognosis.
Children with an inherited genetic syndrome termed Neurofibromatosis Type I are at higher risk of developing tumors of glial origin, including cerebellar low-grade gliomas. The majority of children with cerebellar JPAs developed these tumors spontaneously and have no identifiable cause for the tumor.
What are the symptoms of Cerebellar Astrocytoma?
Due to the slow growth rate of cerebellar JPAs, children will often have months to years of symptoms before presenting to the doctor. Cerebellar JPAs are located in close proximity to the 4th ventricle, therefore 90% will present with obstructive hydrocephalus (headache, vomiting (often early morning), poor balance and lethargy). Most children have difficulty with balance and coordination. They can also complain of double vision and torticollis (turning the head to one side).
How is Cerebellar Astrocytoma diagnosed?
Diagnostic procedures for a cerebellar JPA include an MRI to better define the location of the tumor. A surgical biopsy is required to confirm the diagnosis.
How is Cerebellar Astrocytoma treated?
The initial treatment for cerebellar JPA is surgery, with the goal to completely remove the tumor. The majority of JPAs are able to be completely removed safety. Complete tumor removal is associated with a very high rate of cure. In cases where the tumor recurs after initial complete surgery, or if the tumor re-grows after partial surgical removal, the recommended treatment would be a second attempt at surgical removal of the tumor. Although cerebellar JPAs are responsive to chemotherapy and radiation therapy, it is extremely rare for a cerebellar JPA to require any treatment other than surgical removal. The majority of patients treated for a cerebellar JPA are cured of their disease following the first surgery.
About treatment for Cerebellar Astrocytoma at Children’s Minnesota
Our cancer and blood disorders program consistently achieves excellent results ranking it in the top ten programs in the United States. Children’s Minnesota treats the majority of children with cancer and blood disorders in Minnesota and provides patients access to a variety of clinical trials using ground-breaking new treatments. Through our renowned program, patients experience unparalleled family support, a nationally recognized pain management team, and compassionate, coordinated care.
If you are a family member looking for a Children’s Minnesota neuro-oncologist, please call our clinic at 612-813-5940.
If you are a health professional looking for consultation or referral information, please call Children’s Physician Access at 1-866-755-2121 (toll-free) and ask for the on-call hematologist/oncologist.
Additional information on Cerebellar Astrocytoma
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