Craniopharyngioma Information & Craniopharyngioma Links at HealthHaven.com

Craniopharyngioma
	Classification and external resources
	; Craniopharyngioma
ICD-10	D44.4
ICD-9	237.0
ICD-O:	9350/1
DiseasesDB	3153
MedlinePlus	000345
eMedicine	radio/196
MeSH	D003397

Craniopharyngioma is a type of tumor derived from pituitary gland tissue,^[1] that occurs in children and men and women in their 50s and 60s. ^[2] It Arises from rests of odontogenic epithelium within the suprasellar/diencephalic region .It Contains the deposits of Calcium evident on X-Ray. Histologically resembles the Adamantinoma (The most common Tumor of the tooth). Prognosis : Benign but tends to recur after resection. Patient may present with Bi-temporal Hemianopia as the tumor may press the Optic Chiasma.

It has a point prevalence of approximately 2/100,000.^[3]

They are also known as Rathke pouch tumors, hypophyseal duct tumors, or adamantinomas.

[edit] Presentation

They are very slow growing tumors. They arise from the cells along the pituitary stalk. They are classified as benign.^[4]

Craniopharyngioma is a rare, usually suprasellar^[5] neoplasm, which may be cystic, that develops from the nests of epithelium derived from Rathke's pouch.^[6] Rathke's pouch is an embryonic precursor of the anterior pituitary.

Two distinct types are recognized: adamantinomatous craniopharyngioma and papillary craniopharyngioma. ^[7]^[8]

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. In addition to a long list of possible symptoms, the most common presentation include: headaches, growth failure, and bitemporal heteronymous hemianopsia.

TREATMENT: Consists of subfrontal or transsphenoidal excision (with adjuvant radiotherapy, if total removal is not possible).

[edit] Histology

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.

[edit] Possible symptoms

[edit] Additional images

Enhanced T1 weighted MRI's of craniopharyngiomas.

[edit] See also

Rathke's cleft cyst

[edit] References

^ craniopharyngioma at Dorland's Medical Dictionary
^ Hamid R, Sarkar S, Hossain MA, Mazumder U, Akanda NI, Parvin R (2007). "Clinical picture of craniopharyngioma in childhood". Mymensingh medical journal : MMJ 16 (2): 123–6. PMID 17703145.
^ Garnett MR, Puget S, Grill J, Sainte-Rose C (2007). "Craniopharyngioma". Orphanet journal of rare diseases 2: 18. doi:10.1186/1750-1172-2-18. PMID 17425791.
^ 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.
^ "Endocrine Pathology". http://library.med.utah.edu/WebPath/ENDOHTML/ENDO115.html. Retrieved 2009-05-08.
^ Sekine S, Shibata T, Kokubu A, et al (December 2002). "Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations". Am. J. Pathol. 161 (6): 1997–2001. PMID 12466115. PMC 1850925. http://ajp.amjpathol.org/cgi/pmidlookup?view=long&pmid=12466115.
^ Sekine S, Takata T, Shibata T, et al (December 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. http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0309-0167&date=2004&volume=45&issue=6&spage=573.

[edit] External links

[0] craniopharyngioma at Dorland's Medical Dictionary

[pmid17703145-1] Hamid R, Sarkar S, Hossain MA, Mazumder U, Akanda NI, Parvin R (2007). "Clinical picture of craniopharyngioma in childhood". Mymensingh medical journal : MMJ 16 (2): 123–6. PMID 17703145.

[pmid17425791-2] Garnett MR, Puget S, Grill J, Sainte-Rose C (2007). "Craniopharyngioma". Orphanet journal of rare diseases 2: 18. doi:10.1186/1750-1172-2-18. PMID 17425791.

[pmid17630614-3] 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.

[pmid17592268-4] 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.

[urlEndocrine_Pathology-5] "Endocrine Pathology". http://library.med.utah.edu/WebPath/ENDOHTML/ENDO115.html. Retrieved 2009-05-08.

[pmid12466115-6] Sekine S, Shibata T, Kokubu A, et al (December 2002). "Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations". Am. J. Pathol. 161 (6): 1997–2001. PMID 12466115. PMC 1850925. http://ajp.amjpathol.org/cgi/pmidlookup?view=long&pmid=12466115.

[pmid15569047-7] Sekine S, Takata T, Shibata T, et al (December 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. http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0309-0167&date=2004&volume=45&issue=6&spage=573.

[1]

[2]

[3]

[4]

[5]

[6]

[7]

[8]

Contents