Optic and hypothalamic gliomas

Optic and hypothalamic gliomas are types of brain tumours in children. Both are a type of low-grade glioma. They are usually benign, slow-growing tumours.

Optic gliomas start along the optic nerves. These nerves go from the eyes to the brain. They are the main nerves for sight.

Hypothalamic gliomas start in the hypothalamus.

The main goal of treatment is to save the child’s sight or hormone functions. Treatment for optic and hypothalamic gliomas depends on the:

• child’s age and general health
• location and size of the tumour
• extent of vision loss or other symptoms

Newly diagnosed optic or hypothalamic gliomas may be treated with surgery, radiation therapy, targeted therapy, or chemotherapy.

Children with neurofibromatosis type 1 (NF1) often have tumours that grow slowly. If the tumour is not growing, it may be monitored with active surveillance.

Treatment will be given if the tumour is large, if it is growing, if there is a threat to the child’s sight or if symptoms are getting worse.

Children younger than 1 year of age often have a more aggressive tumour and require more urgent treatment.

Recurrent optic or hypothalamic gliomas have come back after treatment. Treatment options for recurrent optic or hypothalamic glioma will depend on previous treatments used and how your child responded to these treatments. Chemotherapy is commonly used, and radiation therapy is rarely used, to treat recurrent optic or hypothalamic gliomas.

Surgery is only used if the tumour can be reached easily and safely removed or if symptoms are getting worse. Surgery for optic and hypothalamic tumours is difficult as the tumours are often hard to reach. They often invade the nerve leading to the eye, and surgery can result in vision loss in one or both eyes. Surgery may also be used to relieve a buildup of cerebrospinal fluid (CSF).

Find out more about surgery for childhood brain and spinal cord tumours.

Radiation therapy uses high-energy rays or particles to destroy cancer cells. It can shrink optic or hypothalamic tumours or slow their growth. In children younger than 3 years of age, radiation therapy can be damaging to developing brain cells. In these cases, chemotherapy may be given first, followed by radiation therapy, if needed, once the child is older than 3 years of age.

Find out more about radiation therapy for childhood brain and spinal cord tumours.

Chemotherapy uses drugs to destroy cancer cells. It can shrink optic and hypothalamic tumours and delay the need for radiation therapy in young children.

Chemotherapy treatment usually lasts for a year or longer. It is often given as an outpatient treatment, which means that your child will not have to stay overnight.

The most common chemotherapy drugs used are:

• vinblastine
• vincristine
• carboplatin

Find out more about chemotherapy for childhood brain and spinal cord tumours.

Targeted therapy uses drugs to target specific molecules (such as proteins) on cancer cells or inside them to stop the growth and spread of cancer. Targeted therapy is sometimes used to treat optic and hypothalamic gliomas.

Trametinib (Mekinist) is a MEK inhibitor may be used to treat optic and hypothalamic gliomas. It is given in combination with the BRAF inhibitor dabrafenib (Tafinlar).

Find out more about targeted therapy for childhood brain and spinal cord tumours.

Active surveillance may be an option if an optic or hypothalamic tumour is not growing and there are no symptoms. The healthcare team will use MRIs to watch the child closely for any signs that the tumour is growing. Other treatments are started when the tumour progresses.

Children with cancer may be treated in a clinical trial. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.