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{{Craniopharyngioma}}
{{Craniopharyngioma}}
{{CMG}}{{AE}}{{SR}}
{{CMG}}{{AE}}{{Marjan}}
==Overview==
==Overview==
There are two subtypes of craniopharyngioma based on the histological and imaging differences. The two subtypes are adamantinomatous and papillary. Mixed variety is also seen in 15% of cases. Former is predominantly seen in children, and latter in adults. Adamantinomatous type consists of reticular epithelial cells, is cystic, has wet [[keratin]] nodules, calcified. Papillary type consists of metaplastic squamous cells, more solid than [[cystic]], with no wet keratin nodules and calcification is rare.
There are two subtypes of craniopharyngioma based on the [[Histology|histological]] and [[Imaging|imaging features]]: adamantinomatous and papillary.


==Classification==
==Classification==
Craniopharyngiomas are believed to derive from Rathke cleft rather than [[squamous cell]] rests along the craniopharyngeal duct as was previously thought. Craniopharyngiomas are classified according to their histologic appearance. The histological appearances of the two pathological subtypes are different, accounting for the different ''imaging features''.These are said to differ not only in appearances, but also in prognosis and epidemiology.<ref>Histologic classification of Craniopharyngioma. Dr Dylan Kurda and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/craniopharyngioma</ref><ref>Classification of Craniopharyngioma. Cancer gov. http://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq#link/_40_toc</ref>
* Craniopharyngiomas are believed to be derive from [[Rathke's pouch|Rathke cleft]] rather than [[squamous cell]] rests. <ref name="pmid27981519">{{cite journal |vauthors=Mortini P |title=Craniopharyngiomas: a life-changing tumor |journal=Endocrine |volume=57 |issue=2 |pages=191–192 |date=August 2017 |pmid=27981519 |doi=10.1007/s12020-016-1192-2 |url=}}</ref>
 
* Craniopharyngiomas are classified according to their [[Histology|histologic appearance]].  
* The [[Histology|histological appearances]] of the two [[Pathology|pathological subtypes]] are different, accounting for the different imaging features.
* The subtypes are said to differ not only in appearances, but also in prognosis and [[epidemiology]].<ref>Classification of Craniopharyngioma. Cancer gov. http://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq#link/_40_toc</ref>
 
 
*'''Adamantinomatous'''
*'''Adamantinomatous'''
**This type is seen predominantly in '''''children'''''
{| align="right"
**It consists of reticular epithelial cells which have appearances reminiscent of the [[enamel]] pulp of developing teeth
|[[File:Adamantinomatous craniopharyngioma.jpeg|thumb|none|300px|Microscopic pathology of Adamantinomatous craniopharyngioma.[https://commons.wikimedia.org/wiki/File:Adamantinomatous_craniopharyngioma_-_high_mag.jpg Source:Wikimedia Commons] ]]
**There may be single or multiple [[cysts]] filled with thick oily fluid high in protein, blood products, and/or cholesterol, creating the so called "machinery oil"
|}
**"Wet [[keratin]] nodules" are a characteristic histological feature
**This type is seen predominantly in children.<ref name="pmid27981519">{{cite journal |vauthors=Mortini P |title=Craniopharyngiomas: a life-changing tumor |journal=Endocrine |volume=57 |issue=2 |pages=191–192 |date=August 2017 |pmid=27981519 |doi=10.1007/s12020-016-1192-2 |url=}}</ref>
**[[Calcification]] is usually present: 90%
**It consists of [[Reticular|reticular epithelial]] cells which have appearances reminiscent of the [[enamel]] [[Pulp (tooth)|pulp]] of developing teeth.
**''More'' locally aggressive
**There may be single or multiple [[cysts]] filled with thick oily fluid high in [[protein]], [[Blood product|blood products]], and/or [[cholesterol]], creating the so called "machinery oil".<ref name="pmid27604996">{{cite journal |vauthors=Müller HL |title=Risk-adapted, long-term management in childhood-onset craniopharyngioma |journal=Pituitary |volume=20 |issue=2 |pages=267–281 |date=April 2017 |pmid=27604996 |doi=10.1007/s11102-016-0751-0 |url=}}</ref>
**''Higher'' rate of recurrence
**"Wet [[keratin]] [[Nodule (medicine)|nodules]]" are a characteristic [[Histology|histological feature]].<ref name="pmid1260697">{{cite journal |vauthors=Petito CK, DeGirolami U, Earle KM |title=Craniopharyngiomas: a clinical and pathological review |journal=Cancer |volume=37 |issue=4 |pages=1944–52 |date=April 1976 |pmid=1260697 |doi= |url=}}</ref>
**Activating ''beta-catenin'' gene mutations are found in adamantinomatous tumors
**[[Calcification]] is usually present in 90% of the cases.
**They are more [[Vascular tumor|locally aggressive]].
**It has higher rate of [[Recurrence period density entropy|recurrence]].
**Activating [[beta-catenin]] [[gene]] [[Mutation|mutations]] are found in [[Craniopharyngioma|adamantinomatous tumors]].<ref name="pmid10690718">{{cite journal |vauthors=Duff J, Meyer FB, Ilstrup DM, Laws ER, Schleck CD, Scheithauer BW |title=Long-term outcomes for surgically resected craniopharyngiomas |journal=Neurosurgery |volume=46 |issue=2 |pages=291–302; discussion 302–5 |date=February 2000 |pmid=10690718 |doi= |url=}}</ref>
 
 
*'''Papillary'''
*'''Papillary'''
**The papillary subtype is seen almost exclusively in '''''adults'''''
{| align="right"
**It is formed of masses of metaplastic [[squamous cells]]
|[[File:Papillary craniopharyngioma histology.jpg|thumb|none|300px|Microscopic pathology of Papillary Craniopharyngioma [https://upload.wikimedia.org/wikipedia/commons/2/26/Papillary_craniopharyngioma_Histology.jpg Source:Wikimedia Commons] ]]
**"Wet [[keratin]]" nodule is absent
|}
**''[[Cysts]]'' do form, but these are less of a feature, and the tumor is more ''solid''
**The [[Papilla|papillary]] subtype is seen almost exclusively in adults.<ref name="pmid27981519">{{cite journal |vauthors=Mortini P |title=Craniopharyngiomas: a life-changing tumor |journal=Endocrine |volume=57 |issue=2 |pages=191–192 |date=August 2017 |pmid=27981519 |doi=10.1007/s12020-016-1192-2 |url=}}</ref>
**[[Calcification]] is uncommon or even rare
**It is formed of [[Metaplasticity|masses of metaplastic]] [[squamous cell]]s.<ref name="pmid1260697">{{cite journal |vauthors=Petito CK, DeGirolami U, Earle KM |title=Craniopharyngiomas: a clinical and pathological review |journal=Cancer |volume=37 |issue=4 |pages=1944–52 |date=April 1976 |pmid=1260697 |doi= |url=}}</ref>
**''Less'' locally aggressive
 
**''Lower'' rate of recurrence
**"Wet [[keratin]]" [[Nodule (medicine)|nodule]] is absent.<ref name="pmid9761047">{{cite journal |vauthors=Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM |title=The descriptive epidemiology of craniopharyngioma |journal=J. Neurosurg. |volume=89 |issue=4 |pages=547–51 |date=October 1998 |pmid=9761047 |doi=10.3171/jns.1998.89.4.0547 |url=}}</ref>
**''BRAF'' V600E [[mutation]]s are observed in [[papillary]] craniopharyngiomas
**[[Cysts]] do form, but these are less of a feature, and the [[Tumor|tumor is more solid]].<ref name="pmid27604996">{{cite journal |vauthors=Müller HL |title=Risk-adapted, long-term management in childhood-onset craniopharyngioma |journal=Pituitary |volume=20 |issue=2 |pages=267–281 |date=April 2017 |pmid=27604996 |doi=10.1007/s11102-016-0751-0 |url=}}</ref>
**[[Calcification]] is uncommon or even rare.<ref name="pmid1260697">{{cite journal |vauthors=Petito CK, DeGirolami U, Earle KM |title=Craniopharyngiomas: a clinical and pathological review |journal=Cancer |volume=37 |issue=4 |pages=1944–52 |date=April 1976 |pmid=1260697 |doi= |url=}}</ref>
**They are less [[Vascular tumor|locally aggressive]].
**It has lower rate of [[Recurrence period density entropy|recurrence]].
**[[BRAF|BRAF V600E]] [[mutation]]s are observed in [[papillary]] craniopharyngiomas.
*'''Mixed'''
*'''Mixed'''
**15%, but shares imaging features and prognosis similar to ''adamantinomatous''
**15% of them share imaging features and [[prognosis]] similar to the adamantinomatous subtye.<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 |year=2004 |month=December |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>


==References==
==References==
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

Overview

There are two subtypes of craniopharyngioma based on the histological and imaging features: adamantinomatous and papillary.

Classification


  • Adamantinomatous
Microscopic pathology of Adamantinomatous craniopharyngioma.Source:Wikimedia Commons


  • Papillary
Microscopic pathology of Papillary Craniopharyngioma Source:Wikimedia Commons

References

  1. ↑ 1.0 1.1 1.2 Mortini P (August 2017). "Craniopharyngiomas: a life-changing tumor". Endocrine. 57 (2): 191–192. doi:10.1007/s12020-016-1192-2. PMID 27981519.
  2. ↑ Classification of Craniopharyngioma. Cancer gov. http://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq#link/_40_toc
  3. ↑ 3.0 3.1 Müller HL (April 2017). "Risk-adapted, long-term management in childhood-onset craniopharyngioma". Pituitary. 20 (2): 267–281. doi:10.1007/s11102-016-0751-0. PMID 27604996.
  4. ↑ 4.0 4.1 4.2 Petito CK, DeGirolami U, Earle KM (April 1976). "Craniopharyngiomas: a clinical and pathological review". Cancer. 37 (4): 1944–52. PMID 1260697.
  5. ↑ Duff J, Meyer FB, Ilstrup DM, Laws ER, Schleck CD, Scheithauer BW (February 2000). "Long-term outcomes for surgically resected craniopharyngiomas". Neurosurgery. 46 (2): 291–302, discussion 302–5. PMID 10690718.
  6. ↑ Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM (October 1998). "The descriptive epidemiology of craniopharyngioma". J. Neurosurg. 89 (4): 547–51. doi:10.3171/jns.1998.89.4.0547. PMID 9761047.
  7. ↑ 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)


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