Craniopharyngioma classification: Difference between revisions
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==Overview== | ==Overview== | ||
There are two subtypes of craniopharyngioma based on the histological and imaging features: Adamantinomatous and papillary. | 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. <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 believed to 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 histologic appearance. | * Craniopharyngiomas are classified according to their [[Histology|histologic appearance]]. | ||
* The histological appearances of the two pathological subtypes are different, accounting for the different imaging features. | * The histological appearances of the two [[Pathology|pathological subtypes]] are different, accounting for the different imaging features. | ||
* The sub types 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> | * The sub types 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> | ||
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**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> | **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> | ||
**It consists of reticular epithelial cells which have appearances reminiscent of the [[enamel]] pulp of developing teeth. | **It consists of [[Reticular|reticular epithelial]] cells which have appearances reminiscent of the [[enamel]] [[Pulp (tooth)|pulp]] of developing teeth. | ||
**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".<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> | **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".<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> | ||
**"Wet [[keratin]] nodules" are a characteristic histological feature. | **"Wet [[keratin]] nodules" are a characteristic histological feature. | ||
**[[Calcification]] is usually present | **[[Calcification]] is usually present in 90% | ||
**They are | **They are more locally aggressive. | ||
**It has higher rate of recurrence. | **It has higher rate of recurrence. | ||
**Activating beta-catenin gene mutations are found in adamantinomatous tumors. | **Activating beta-catenin [[gene]] mutations are found in adamantinomatous tumors. | ||
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**It is formed of masses of metaplastic [[squamous cell]]s. | **It is formed of masses of metaplastic [[squamous cell]]s. | ||
**"Wet [[keratin]]" nodule is absent. | **"Wet [[keratin]]" nodule is absent. | ||
** | **[[Cysts]] do form, but these are less of a feature, and the 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. | **[[Calcification]] is uncommon or even rare. | ||
**They are less locally aggressive. | **They are less locally aggressive. | ||
**It has | **It has lower rate of recurrence. | ||
**BRAF V600E [[mutation]]s are observed in [[papillary]] craniopharyngiomas. | **BRAF V600E [[mutation]]s are observed in [[papillary]] craniopharyngiomas. | ||
*'''Mixed''' | *'''Mixed''' |
Revision as of 00:25, 15 February 2019
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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
- Craniopharyngiomas are believed to derive from Rathke cleft rather than squamous cell rests. [1]
- Craniopharyngiomas are classified according to their histologic appearance.
- The histological appearances of the two pathological subtypes are different, accounting for the different imaging features.
- The sub types are said to differ not only in appearances, but also in prognosis and epidemiology.[2]
- Adamantinomatous
- This type is seen predominantly in children.[1]
- It consists of reticular epithelial cells which have appearances reminiscent of the enamel pulp of developing teeth.
- 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".[3]
- "Wet keratin nodules" are a characteristic histological feature.
- Calcification is usually present in 90%
- They are more locally aggressive.
- It has higher rate of recurrence.
- Activating beta-catenin gene mutations are found in adamantinomatous tumors.
- Papillary
- The papillary subtype is seen almost exclusively in adults.[1]
- It is formed of masses of metaplastic squamous cells.
- "Wet keratin" nodule is absent.
- Cysts do form, but these are less of a feature, and the tumor is more solid.[3]
- Calcification is uncommon or even rare.
- They are less locally aggressive.
- It has lower rate of recurrence.
- BRAF V600E mutations are observed in papillary craniopharyngiomas.
- Mixed
- 15%, but shares imaging features and prognosis similar to adamantinomatous.
References
- ↑ 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.
- ↑ Classification of Craniopharyngioma. Cancer gov. http://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq#link/_40_toc
- ↑ 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.