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Two distinct types are recognized:<ref name="pmid12466115">{{cite journal |author=Sekine S, Shibata T, Kokubu A, ''et al'' |title=Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations |journal=Am. J. Pathol. |volume=161 |issue=6 |pages=1997–2001 |date=December 2002 |pmid=12466115 |pmc=1850925 |doi= |url=http://ajp.amjpathol.org/cgi/pmidlookup?view=long&pmid=12466115}}</ref><ref name="pmid15569047">{{cite journal |author=Sekine S, Takata T, Shibata T, ''et al'' |title=Expression of enamel proteins and LEF1 in adamantinomatous craniopharyngioma: evidence for its odontogenic epithelial differentiation |journal=Histopathology |volume=45 |issue=6 |pages=573–9 |date=December 2004 |pmid=15569047 |doi=10.1111/j.1365-2559.2004.02029.x |url=http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0309-0167&date=2004&volume=45&issue=6&spage=573}}</ref>
Two distinct types are recognized:<ref name="pmid12466115">{{cite journal |author=Sekine S, Shibata T, Kokubu A, ''et al'' |title=Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations |journal=Am. J. Pathol. |volume=161 |issue=6 |pages=1997–2001 |date=December 2002 |pmid=12466115 |pmc=1850925 |doi= |url=http://ajp.amjpathol.org/cgi/pmidlookup?view=long&pmid=12466115}}</ref><ref name="pmid15569047">{{cite journal |author=Sekine S, Takata T, Shibata T, ''et al'' |title=Expression of enamel proteins and LEF1 in adamantinomatous craniopharyngioma: evidence for its odontogenic epithelial differentiation |journal=Histopathology |volume=45 |issue=6 |pages=573–9 |date=December 2004 |pmid=15569047 |doi=10.1111/j.1365-2559.2004.02029.x |url=http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0309-0167&date=2004&volume=45&issue=6&spage=573}}</ref>
*Adamantinomatous craniopharyngioma, which resemble [[adamantinoma]]s, the most common type of tooth tumor; and,
*Adamantinomatous craniopharyngioma, which resemble [[adamantinoma]]s, the most common type of tooth tumor characterized by activating CTNNB1 mutations; and,
*Papillary craniopharyngioma <ref>characterized by BRAFv600E mutations</ref><ref>{{cite journal|url=http://www.nature.com/ng/journal/v46/n2/full/ng.2868.html}}</ref> .
*Papillary craniopharyngioma.


In the adamantinomatous type, calcifications are visible on neuroimaging and are helpful in diagnosis.
In the adamantinomatous type, calcifications are visible on neuroimaging and are helpful in diagnosis.
The papillary type rarely calcifies.
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]].
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]].

Revision as of 00:22, 10 April 2014

Craniopharyngioma
SpecialtyOncology Edit this on Wikidata

Craniopharyngioma is a type of brain tumor derived from pituitary gland embryonic tissue,[1] that occurs most commonly in children but also in men and women in their 50s and 60s.[2] Patients may present with bitemporal inferior quadrantanopia leading to bitemporal hemianopia, as the tumor may compress the optic chiasm.

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

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

Presentation

CT scan showing a craniopharyngioma.

Craniopharyngioma is a rare, usually suprasellar[4] neoplasm, which may be cystic, that develops from nests of epithelium derived from Rathke's pouch.[5][6] 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, which are evident on an x-ray. They are classified by histology as benign;[7] however, as with many brain tumors, their treatment can be difficult, and significant morbidities are associated with both the tumor and treatment.

Histology

Micrograph showing the characteristic features of an adamantinomatous craniopharyngioma - cystic spaces, calcifications, and "wet" keratin. HPS stain.
Micrograph showing a papillary craniopharyngioma. HPS stain.

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:[8][9]

  • Adamantinomatous craniopharyngioma, which resemble adamantinomas, the most common type of tooth tumor characterized by activating CTNNB1 mutations; and,
  • Papillary craniopharyngioma [10][11] .

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.

Prognosis

Craniopharyngiomas are generally benign but are known to recur after resection.

Treatment

Treatment generally consists of subfrontal or transsphenoidal excision. Surgery using the transsphenoidal route is often performed by a joint team of ENT and neurosurgeons. Because of the location of the craniopharyngioma near the brain and skullbase, a surgical navigation system might be used to verify the position of surgical tools during the operation.[12] Adjuvant radiotherapy is also used if total removal is not possible. Due to the morbidities 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 frequently employed, especially in young patients. The tumor, being in the pituitary gland, can cause secondary health problems. The immune system, thyroid levels, growth hormone levels and testosterone levels can be compromised from craniopharygioma. All of the before mentioned health problems can be treated with modern medicine.[13]

Possible symptoms

Additional images

  • Enhanced T1 weighted MRI's of craniopharyngiomas.
    Enhanced T1 weighted MRI's of craniopharyngiomas.

See also

References

  1. ^ "craniopharyngioma" at Dorland's Medical Dictionary[dead link]
  2. ^ 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.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  3. ^ 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. PMC 1855047. PMID 17425791.{{cite journal}}: CS1 maint: multiple names: authors list (link) CS1 maint: unflagged free DOI (link)
  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.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  5. ^ Moore, Kraig D.; Couldwell, William T. (January 15, 2000). "41. Craniopharyngioma". In Bernstein, Mark; Berger, Mitchel S. (eds.). Neuro-oncology: the essentials. Endorsed by the Joint Tumor Section of the American Association of Neurological Surgeons & the Congress of Neurological Surgeons. Thieme. pp. 409–418. ISBN 978-0-86577-880-1. Retrieved August 8, 2011. {{cite book}}: External link in |chapterurl= (help); Unknown parameter |chapterurl= ignored (|chapter-url= suggested) (help)
  6. ^ "Endocrine Pathology". Retrieved 2009-05-08.
  7. ^ 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.
  8. ^ 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. PMC 1850925. PMID 12466115. {{cite journal}}: Explicit use of et al. in: |author= (help)CS1 maint: multiple names: authors list (link)
  9. ^ 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. {{cite journal}}: Explicit use of et al. in: |author= (help)CS1 maint: multiple names: authors list (link)
  10. ^ characterized by BRAFv600E mutations
  11. ^ http://www.nature.com/ng/journal/v46/n2/full/ng.2868.html. {{cite journal}}: Cite journal requires |journal= (help); Missing or empty |title= (help)
  12. ^ Use of surgical navigation for craniopharyngioma removal [dead link]
  13. ^ Wisoff JH (February 2008). "Craniopharyngioma". J Neurosurg Pediatr. 1 (2): 124–5, discussion 125. doi:10.3171/PED/2008/1/2/124. PMID 18352780.

External links

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