Craniopharyngioma pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Pathophysiology
They are very slow growing tumors. They arise from the cells along the pituitary stalk. They are classified as benign.[1]
Craniopharyngioma is a rare, usually suprasellar[2] neoplasm, which may be cystic, that develops from the nests of epithelium derived from Rathke's pouch.
Gross Pathology
On macroscopic examination, craniopharyngiomas are cystic or partially cystic with solid areas.
Microscopic pathology
The histologic pattern consists of nesting of squamous epithelium bordered by radially arranged cells. It is frequently accompanied by calcium deposition and have a microscopic papillary architecture. Two distinct types are recognized:[3][4]
- Adamantinomatous craniopharyngioma and,
- Papillary craniopharyngioma.
In the adamantinomatous type, calcifications are visible on neuroimaging and are helpful in diagnosis. The papillary type rarely calcifies. 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. In addition to a long list of possible symptoms, the most common presentation include: headaches, growth failure, and bitemporal hemianopsia.
Shown below is a micrograph showing the characteristic features of an adamantinomatous craniopharyngioma - cystic spaces, calcifications, and "wet" keratin. HPS stain.
Shown below is a Micrograph showing a papillary craniopharyngioma. HPS stain.
References
- ↑ Garrè ML, Cama A (2007). "Craniopharyngioma: modern concepts in pathogenesis and treatment". Curr. Opin. Pediatr. 19 (4): 471–9. doi:10.1097/MOP.0b013e3282495a22. PMID 17630614.
- ↑ Rodriguez FJ, Scheithauer BW, Tsunoda S, Kovacs K, Vidal S, Piepgras DG (2007). "The spectrum of malignancy in craniopharyngioma". Am. J. Surg. Pathol. 31 (7): 1020–8. doi:10.1097/PAS.0b013e31802d8a96. PMID 17592268.
- ↑ Sekine S, Shibata T, Kokubu A; et al. (2002). "Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations". Am. J. Pathol. 161 (6): 1997–2001. PMC 1850925. PMID 12466115. Unknown parameter
|month=ignored (help) - ↑ Sekine S, Takata T, Shibata T; et al. (2004). "Expression of enamel proteins and LEF1 in adamantinomatous craniopharyngioma: evidence for its odontogenic epithelial differentiation". Histopathology. 45 (6): 573–9. doi:10.1111/j.1365-2559.2004.02029.x. PMID 15569047. Unknown parameter
|month=ignored (help)