Craniopharyngiomas are histologically benign (non-cancerous), slow-growing tumours that grow out of embryologic remnants that were misplaced during the formation of the pituitary gland. They occur exclusively in the region of the pituitary gland.
Craniopharyngiomas account for approximately 2 – 3% of all intracranial neoplasms and are most common in childhood and adolescence and they represent 5 – 13% of all brain tumours occurring in children. There is no sexual predisposition. Despite its histologic appearance, craniopharyngiomas may rarely behave like malignant tumours and can metastasize.
Craniopharyngiomas may be intrasellar or suprasellar and may cause various degrees of hypopituitarism by compression of the anterior part of the pituitary gland, pituitary stalk or the hypothalamus. Craniopharyngiomas are uniformly benign and they do not produce hormones. They can be solid, cystic or mixed, they may show various degrees of calcification. Due to their slow-growing characteristics, these tumours can take years to manifest themselves before a diagnosis is made.
The symptoms associated with a craniopharyngioma are related to its location and size. They include symptoms related to an increase in the intracranial pressure (ICP) due to blockage of cerebrospinal fluid (CSF) pathways causing headache, nausea, vomiting and difficulty with balance; and symptoms related to direct pressure they exert on the surrounding neurovascular structures such as decreased vision, particularly peripheral vision due to direct pressure on the optic nerves. They may also cause hormonal manifestations due to pressure on the hypothalamus and pituitary gland causing obesity, excessive thirst and urination (diabetes insipidus), stunted growth and delayed developmental milestones.
The treatment of choice for craniopharyngiomas is surgical removal. However, radiation therapy (conventional or gamma knife (GK)) may be combined especially in cases of residual tumour following surgery. If possible, radiation is withheld in younger children to prevent their delayed side effects.
In general, the prognosis is good, but it depends on several factors, including the ability of the tumour to be completely removed, and the neurological deficits and hormonal imbalances caused by the tumor at time of treatment.