Craniopharyngioma
| Craniopharyngioma | |
|---|---|
 |
|
| Micrograph of a craniopharyngioma (adamantinomatous). HPS stain. | |
| Classification and external resources | |
| Specialty | oncology |
| ICD-10 | D44.4 |
| ICD-9-CM | 237.0 |
| ICD-O | 9350/1 |
| DiseasesDB | 3153 |
| MedlinePlus | 000345 |
| eMedicine | radio/196 |
| Patient UK | Craniopharyngioma |
| MeSH | D003397 |
Craniopharyngioma is a type of brain tumor derived from pituitary gland embryonic tissue, that occurs most commonly in children but also in men and women in their 50s and 60s. People may present with bitemporal inferior quadrantanopia leading to bitemporal hemianopsia, as the tumor may compress the optic chiasm.
It has a point prevalence of approximately 2/100,000.
Craniopharyngiomas are also known as Rathke pouch tumors, hypophyseal duct tumors, or adamantinomas.
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. They are classified by histology as benign; however, as with many brain tumors, their treatment can be difficult, and significant morbidities are associated with both the tumor and treatment.
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.
Two distinct types are recognized:
In the adamantinomatous type, calcifications are visible on neuroimaging and are helpful in diagnosis. The papillary type rarely calcifies.
On macroscopic examination, craniopharyngiomas are cystic or partially cystic with solid areas. On light microscopy, the cysts are seen to be lined by stratified squamous epithelium. Keratin pearls may also be seen. The cysts are usually filled with a yellow, viscous fluid which is rich in cholesterol crystals. Of a long list of possible symptoms, the most common presentations include: headaches, growth failure, and bitemporal hemianopsia.
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Wikipedia