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Craniopharyngioma

Craniopharyngioma is a rare tumor in the brain, in the pituitary gland. The pituitary gland is located in the cranial base and is connected to the hypothalamus via the pituitary stalk. The pituitary gland and hypothalamus control the production and release of different hormones.

A craniopharyngioma may occur in the cranial base at the site of the pituitary gland or just above the cranial base along the pituitary stalk to the hypothalamus.  They are benign tumors that often grow slowly and are often accompanied by the formation of cysts (blisters containing fluid).


Symptoms of this disease

When it grows, the tumor can quickly cause pressure at the crossing of the optic nerves (optic chiasm). As growth continues, expansion towards the hypothalamus or third chamber of the brain (ventricle) may also occur. Sometimes this leads to a closure of normal cerebrospinal fluid circulation, resulting in a hydrocephalus. The patient may then complain of headaches, nausea, vomiting and increased drowsiness.

The location of the tumor, in the pituitary gland, can lead to endocrine disruption, such as late puberty. These hormone disturbances can sometimes remain unnoticed for a long time.

 

How do we make the diagnosis?

Initially, a CT scan is performed to see if any deviations are visible. If there is any indication of a tumor in the pituitary gland, an additional examination will be carried out by means of an MRI.
The craniopharyngioma image is very specific, so the diagnosis can most likely be made on the basis of these scans.

 
Behandeling van craniopharyngeoom

The treatment of the tumor itself varies from patient to patient. If the tumor has a large cyst, you can initially choose to control the tumor by administering a medicine (interferon alpha) via a small tube in the cyst.
If there is pressure on the optic nerves, this is followed by an operation that reduces the pressure. This can be done via access through the skull (craniotomy) or sometimes through the nose (endonasal). In the event of an operation in which the tumor is removed, there is a high chance that the pituitary gland will completely fail. This is very radical and requires intensive lifelong support from the pediatric endocrinologist. If the tumor cannot be completely removed or grows again, additional radiotherapy may be necessary.

Craniopharyngiomas can generally be effectively treated, but the consequences, such as hormonal failure, can be significant for a child.