A craniopharyngioma is a rare type of brain tumor that forms near the pituitary gland, a small organ that secretes hormones that regulate many bodily functions. Most craniopharyngiomas are benign (non-cancerous), but they behave aggressively locally and can infiltrate and compress surrounding structures if left untreated. As it develops, the tumor can affect the function of the pituitary gland, the pathways of vision and sometimes the function of the hypothalamus (an important part of the brain). They can also block the circulation of cerebrospinal fluid, leading to hydrocephalus, i.e. dilation of the fluid cavities normally present in the brain.
These tumors are cared for by a multidisciplinary team specialized and experienced in the treatment of these rare tumors, and including neurosurgeons, endocrinologists, ophthalmologists, neuroradiologists and radiotherapists.
Frequency of occurrence
Craniopharyngiomas are very rare, accounting for 2-5% of all primary brain tumors and 5-10% of all childhood brain tumors.
Age of onset
It can occur at any age, even before birth. However, they are more common in children aged between 5 and 14 and adults over 45. Both sexes are equally affected.
Diagnosis
The diagnostic workup for a craniopharyngioma includes:
– a neurological examination,
– imaging, such as CT scans and MRIs,
– blood tests to measure hormone secretion levels.
Following this work-up, the diagnosis is generally made with a high degree of probability, as the appearance and presentation of this tumor are generally very characteristic. The diagnosis will be confirmed by analysis of the tumour removed during surgery.
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Craniopharyngiomas are usually detected when they begin to produce symptoms by pressing on the brain, pituitary gland, blood vessels, optic nerves, or optic chiasma (this is where the two optic nerves leading to the eyes cross just before entering the brain) and the cavities in which cerebrospinal fluid circulates. Symptoms vary considerably depending on the extent of the tumour.
The most common symptoms are :
- Headaches
- Endocrine dysfunction linked to defective hormone secretion
- Vision problems
If the tumor compresses the pituitary gland or pituitary stalk, it may cause the following effects:
– stunted growth,
– delayed puberty,
– loss of normal menstrual function,
– loss of sexual desire (libido),
– increased sensitivity to cold,
– fatigue,
– constipation,
– dry skin,
– nausea,
– low blood pressure,
– depression,
– diabetes insipidus: excessive thirst, excessive urination,
– milk discharge from breasts (galactorrhea) if prolactin levels are increased.
If the tumor compresses the optic nerve or chiasma, it may cause the following effects:
– blurred vision,
– reduced visual field,
– to blindness.
If the tumor compresses the hypothalamus, it can provoke:
– obesity,
– drowsiness,
– abnormalities in body temperature regulation,
– diabetes insipidus,
– mood swings, slowing down,
– headache,
– confusion,
– vomiting.
Surgery is generally recommended to remove as much of the tumor as possible and relieve pressure on the brain, optic pathways and hypothalamus. The tumor can be removed through an opening in the skull (craniotomy) or through the nose using an endoscope and instruments specific to this approach (transsphenoidal surgery). Depending on the extent of the tumor, it may be difficult, if not impossible, to maintain pituitary gland function, necessitating postoperative hormone replacement therapy. This is a delicate surgery involving risks that are discussed with the patient and his or her family prior to the operation, in order to define the most appropriate therapeutic strategy.
If the entire tumour cannot be surgically removed, radiotherapy may be recommended to prevent growth of the tumour residue left after surgery. Nevertheless, the risk of recurrence persists. Radiosurgery may also be proposed for small volumes at a distance from the optic pathways (optic nerves and chiasma).
The department participates in a monthly national multidisciplinary meeting at which all cases are discussed in a collegial manner, in order to make the best possible decision for each individual patient.