Craniopharyngioma

Craniopharyngioma is a rare type of brain tumor derived from pituitary gland embryonic tissue that occurs most commonly in children, but also affects adults. It may present at any age, even in the prenatal and neonatal periods, but peak incidence rates are childhood-onset at 5–14 years and adult-onset at 50–74 years. People may present with bitemporal inferior quadrantanopia leading to bitemporal hemianopsia, as the tumor may compress the optic chiasm. It has a point prevalence around two per 1,000,000. Craniopharyngiomas are distinct from Rathke's cleft tumours and intrasellar arachnoid cysts.CT scan showing a craniopharyngioma.Enhanced T1 weighted MRI's of craniopharyngiomas.Micrograph showing the characteristic features of an adamantinomatous craniopharyngioma - cystic spaces, calcifications, and 'wet' keratin. HPS stain.Micrograph showing a papillary craniopharyngioma. HPS stain. Craniopharyngioma is a rare type of brain tumor derived from pituitary gland embryonic tissue that occurs most commonly in children, but also affects adults. It may present at any age, even in the prenatal and neonatal periods, but peak incidence rates are childhood-onset at 5–14 years and adult-onset at 50–74 years. People may present with bitemporal inferior quadrantanopia leading to bitemporal hemianopsia, as the tumor may compress the optic chiasm. It has a point prevalence around two per 1,000,000. Craniopharyngiomas are distinct from Rathke's cleft tumours and intrasellar arachnoid cysts. Craniopharyngiomas are almost always benign. However, as with many brain tumors, their treatment can be difficult, and significant morbidities are associated with both the tumor and treatment. Craniopharyngioma is a rare, usually suprasellar neoplasm, which may be cystic, that develops from nests of epithelium derived from Rathke's pouch. Rathke's pouch is an embryonic precursor of the anterior pituitary. Craniopharyngiomas are typically very slow growing tumors. They arise from the cells along the pituitary stalk, specifically from nests of odontogenic (tooth-forming) epithelium within the suprasellar/diencephalic region and, therefore, contain deposits of calcium that are evident on an x-ray. There are a few scans and tests that the physician can conduct in order to diagnose a person with craniopharyngioma. High-resolution magnetic resonance imaging (MRI) is valuable because it allows the neuroradiologist to view the tumor from different angles. In some cases, a powerful 3T (Tesla) MRI scanner can help define the location of critical brain structures affected by the tumor.The histologic pattern consists of nesting of squamous epithelium bordered by radially arranged cells. It is frequently accompanied by calcium deposition and may have a microscopic papillary architecture.A computed tomography (CT) scan is also a good diagnostic tool as it detects calcification in the tumor. Two distinct types are recognized: