A craniopharyngioma is a rare type of brain tumor derived from pituitary gland embryonic tissue[1] that occurs most commonly in children, but also affects adults.
Craniopharyngioma is a rare, usually suprasellar[15] neoplasm, which may be cystic, that develops from nests of epithelium derived from Rathke's pouch.
They arise from the cells along the pituitary stalk, specifically from nests of odontogenic (tooth-forming) epithelium within the suprasellar/diencephalic region, so contain deposits of calcium that are evident on an X-ray.
[20] In some cases, a powerful 3T (Tesla) MRI scanner can help define the location of critical brain structures affected by the tumor.
Of a long list of possible symptoms, the most common presentations include headaches, growth failure, and bitemporal hemianopsia.
Due to the poor outcomes associated with damage to the pituitary and hypothalamus from surgical removal and radiation, experimental therapies using intracavitary phosphorus-32, yttrium, or bleomycin delivered via an external reservoir are sometimes employed, especially in young patients.
[31] Proton therapy affords a reduction in dose to critical structures compared to conventional photon radiation, including IMRT, for patients with craniopharyngioma.
[32] The most effective treatment 'package' for the malignant craniopharyngiomas described in literature is a combination 'gross total resective' surgery with adjuvant chemoradiotherapy.
The chemotherapy drugs paclitaxel and carboplatin have shown a clinical (but not statistical) significance in increasing the survival rate in patients who have had gross total resections of their malignant tumours.
[33] Limited surgery minimizing hypothalamic damage may decrease the severe obesity rate at the expense of the need for radiotherapy to complete the treatment.
[34] Role of radiotherapy: Aggressive attempts at total removal do result in prolonged progression-free survival in most patients, but for tumors that clearly involve the hypothalamus, complications associated with radical surgery have prompted to adopt a combined strategy of conservative surgery and radiation therapy to residual tumor with as high a rate of cure.
Radical excision is associated with a risk of mortality or morbidity particularly as hypothalamic damage, visual deterioration, and endocrine complication between 45 and 90% of cases.
Alternative treatments consisting of radiosurgery, intracavitary application of isotopes, and brachytherapy also offer an acceptable tumor control and might be given in selected cases.