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Craniopharyngioma

Every year, approximately 8-10 children in the Netherlands are diagnosed with craniopharyngioma. This brain tumor can occur at any age, but mostly between 5 and 10 years old. A craniopharyngioma is a rare tumor in the brain near the pituitary gland and/or the hypothalamus.

The pituitary gland is located in the brain, just below the junction of the optic nerves. The hypothalamus is above the pituitary gland. It is a small but very important area. The pituitary gland and hypothalamus together regulate hormone balance. This is a complex system that controls a number of important processes in the body, such as activity, hunger and thirst, metabolism, temperature, day and night rhythm, growth, puberty development and the release of stress hormone. Craniopharyngiomas grow slowly and do not spread.

The brochure entitled Low-grade glioma contains comprehensive information about this disease. The most important information is provided below.

Causes

A craniopharyngioma develops in the so-called Rathke's pouch during pregnancy. This pouch normally disappears before birth, but if a few cells of this pouch survive, they can develop into a craniopharyngeoma.

Symptoms

Children with a craniopharyngioma at first have few symptoms, if any. Only when the tumor presses on the surrounding tissue will they suffer vision and hormone problems. Examples here are growth retardation, early puberty, continuous hunger pangs, considerable weight gain, lack of energy, inability to maintain body heat, fluctuations in their salt content due to a disrupted sense of thirst, disturbed day-night rhythm and behavioral problems. In some instances, the cerebrospinal fluid can no longer be removed, causing symptoms such as headache, nausea, vomiting and drowsiness due to increased pressure in the brain.

How is craniopharyngioma diagnosed?

An MRI scan is performed and cerebrospinal fluid is drawn under sedation. In some instances, this is followed by a CT scan.

Treatment

When the pressure in the brain becomes too great, your child is first fitted with a drain. The neurosurgeon inserts a tube into your child's head to drain the fluid and reduce the symptoms. This procedure takes place under anesthesia. If the drain is to remain in place for any length of time, your child is given a shunt, in which case the tube runs down to the abdominal cavity where the fluid is removed by the body. In older children, the neurosurgeon sometimes makes a tiny hole between the ventricles, creating more space and reducing pressure. The treatment of the tumor depends on the symptoms and the characteristics of the tumor shown on the MRI scan. The treatment differs from one child to the other. In the event of pressure on the optic nerves, the neurosurgeon will try to surgically remove the tumor. If the tumor cannot be removed completely, radiation therapy is considered. In children who receive radiotherapy after surgery, the pituitary gland stops working and so they always need replacement hormones. In some children, the residual tumor remains stable for a prolonged period of time and no radiotherapy is required. Sometimes children require a second operation, always followed by radiotherapy.
In the event of a tumor with a large cyst, interferon alpha is administered via an Ommaya reservoir into the cyst several times a week until the tumor is stabilized. All children are regularly checked by means of an MRI scan.

Consequences

Due to the tumor itself, but also because of the surgery and radiotherapy, there is a good chance that the pituitary gland ceases to function properly. In that case, your child will need hormone replacement therapy for the rest of their life under the intensive guidance of the (pediatric) endocrinologist. In many children, the hypothalamus no longer works properly because of the tumor. They might suffer from reduced energy, lack of initiative, temperature problems, sleep disorders and/or become seriously obese due to uncontrollable hunger pangs. This has a huge impact on the life of the child and the family. Many children also suffer permanent visual impairment.

Chance of recovery

In general, craniopharyngiomas can be treated successfully, but the tumor and the treatment can have far-reaching consequences.

Questions?

Children and adolescents with craniopharyngeoma are treated in the neuro-oncology department.