Cerebellar Low-grade Astrocytoma
What is a cerebellar low-grade astrocytoma?
Cerebellar low-grade astrocytomas are grade I or grade II astrocytomas that occur in an area of the brain called the cerebellum. Grade I astrocytomas make up about 80% of low-grade cerebellar astrocytomas.
The cerebellum is located in the posterior fossa and controls movements and balance, so children with cerebellar tumors often have balance and coordination problems. Children with tumors in the posterior fossa also often have increased pressure in the brain that is caused by build-up of cerebrospinal fluid (also called hydrocephalus). Symptoms of increased pressure are headaches, vomiting, and increased sleepiness as well as vision problems.
What causes cerebellar low-grade astrocytomas?
For the majority of cases we do not understand why children develop cerebellar low-grade astrocytomas. There is nothing you could have done to prevent this from happening. There are certain genetic syndromes such as neurofibromatosis or tuberous sclerosis that are associated with astrocytomas, but for the majority of children these tumors occur without identifiable cause.
What is the outcome?
Children with cerebellar low-grade astrocytomas generally have excellent outcomes. Grade I cerebellar astrocytomas have a 10-year progression-free survival of about 80-90% if the tumor was removed completely with surgery. Grade II astrocytomas are relatively rare in children but carry a worse prognosis and often recur after surgery, requiring additional treatment such as chemotherapy and/or radiation.
What is the treatment for cerebellar low-grade astrocytomas?
The mainstay of treatment for low-grade cerebellar astrocytomas is surgery. During surgery, your child’s pediatric neurosurgeon will remove as much of the tumor as possible. Once the tumor is removed, children are often monitored with imaging such as MRI to make sure that the tumor does not come back. If the tumor could not be completely removed surgically or if there is evidence of recurrence, additional treatment is often warranted.
What is the treatment for recurrent or progressive cerebellar low-grade astrocytomas?
Once there is evidence of tumor recurrence there are multiple options for additional treatment such as a second surgery, chemotherapy, and/or radiation therapy. The recommendation for additional treatment will depend on factors such as associated risks of another surgery and the age of your child.
What clinical trials are available for children with low-grade astrocytomas?
There are generally several trials available for children with progressive/recurrent low-grade gliomas. UCSF is part of the Pediatric Brain Tumor Consortium, which is a network of institutions funded by the National Cancer Institute that offers clinical trials of new therapies for children with brain tumors. We also partner with the Children’s Oncology Group and some industry sponsors to provide access to pediatric brain tumor clinical trials.
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 on low-grade astrocytomas?
Over the last few years there has been significant advancement in our understanding of the underlying molecular features that are associated with low-grade gliomas. Researchers have discovered that the majority of cerebellar grade I gliomas carry a mutation called BRAF-KIAA1549 fusion protein. This protein activates certain signaling pathways in the tumor cells and researchers at UCSF are currently investigating inhibitors of this pathway as a potential treatment option.