Craniopharyngioma

Craniopharyngiomas are a type of brain tumour that occurs in children. They are slow-growing, non-cancerous (benign) tumours. They occur in the central part of the brain above the pituitary gland, near the hypothalamus, optic nerve and third ventricle. They can be present at birth (called congenital), but symptoms often don’t appear until the tumour starts to grow and affect other areas of the brain. Craniopharyngiomas are usually part solid tumour and part fluid-filled cyst.

These tumours can affect vision, so your child may have vision tests before treatment begins. Craniopharyngiomas can also affect the pituitary gland and hypothalamus, which can cause hormone problems.

The healthcare team will suggest treatments based on your child's needs and work with you to develop a treatment plan. Treatment of craniopharyngiomas depends on:

• the child’s age and general health
• the size and location of the tumour
• if the tumour extends to or involves nearby important structures such as the pituitary gland, hypothalamus and optic nerve

Treatment often begins with seeing an endocrinologist. An endocrinologist is a doctor who specializes in treating hormone problems. The endocrinologist will assess any damage to the pituitary gland and can give synthetic hormones to replace any hormones that are missing.

Newly diagnosed craniopharyngiomas are often treated with surgery along with radiation therapy. Cysts may need to be drained if they are causing a buildup of cerebrospinal fluid (CSF) in the brain (called hydrocephalus). Chemotherapy, radiation or immunotherapy can be injected into cysts (called intracystic therapy).

Recurrent craniopharyngiomas are tumours that come back after treatment. Treatment will depend on what type of tumour comes back (solid or cystic). Surgery, radiation therapy or both may be used to treat a recurrent solid tumour. Intracystic immunotherapy, chemotherapy or radiation therapy may be used to treat cysts that come back.

Surgery is commonly used to treat craniopharyngiomas. How surgery is done will depend on if the tumour is mostly solid or mostly fluid-filled (cystic). The size of the tumour will also determine if transsphenoidal surgery or a craniotomy is used to remove the tumour.

For solid tumours, the goal is to remove as much of the tumour as possible without affecting brain functions. This can be hard because the tumour is usually close to or attached to vital structures, such as the pituitary gland, hypothalamus and optic nerve. Trying to remove a tumour from these structures would cause more harm than good. Therefore, part of the tumour is usually removed (partial resection) and radiation therapy is given to the area.

For cystic tumours, if one of the cysts is pressing against an important part of the brain and causing increased pressure, the cyst may be drained. This involves either making a small hole in the skull and inserting a tube into the cyst or putting in a shunt called an Ommaya reservoir. Immunotherapy can then be injected directly into the cyst to dry it out, shrink it and prevent any further fluid from forming. The immunotherapy drug interferon-alpha is commonly used.

Surgery may also be used to relieve a buildup of 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 is often given after part of a craniopharyngioma has been removed to try to stop it from growing. This approach may be tried if a partial resection doesn't stop the tumour from coming back and a total resection is too risky or may cause too many side effects. Even if the surgeon can completely remove the tumour, radiation therapy may be given to destroy any cancer cells that may have been left behind.

If a craniopharyngioma has many cysts, radiation may be delivered by inserting radioactive material, such as phosphorous-32, directly into the cyst. This is called intracystic radiation therapy. The cyst receives most of the radiation, and the surrounding tissue is usually spared.

In children younger than 3 years of age, radiation can damage developing brain cells. In these cases, doctors will try to delay giving radiation until the child is older.

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

Immunotherapy helps strengthen or restore the immune system’s ability to fight cancer. It is sometimes used to treat craniopharyngiomas. The immunotherapy drug used is interferon-alpha. It may be given directly into the tumour to delay the need for surgery or radiation therapy.

Find out more about immunotherapy.

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.