Craniopharyngioma classification: Difference between revisions
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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 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> | ||
*'''Adamantinomatous''' | *'''Adamantinomatous''' | ||
**This type is seen predominantly in '''''children''''' | **This type is seen predominantly in '''''children'''''. | ||
**It consists of reticular epithelial cells which have appearances reminiscent of the [[enamel]] pulp of developing teeth | **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" | **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 | **"Wet [[keratin]] nodules" are a characteristic histological feature. | ||
**[[Calcification]] is usually present: 90% | **[[Calcification]] is usually present: 90% | ||
**''More'' locally aggressive | **They are ''More'' locally aggressive. | ||
**''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. | ||
*'''Papillary''' | *'''Papillary''' | ||
**The papillary subtype is seen almost exclusively in '''''adults''''' | **The papillary subtype is seen almost exclusively in '''''adults'''''. | ||
**It is formed of masses of metaplastic [[squamous | **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'' | **''[[Cysts]]'' do form, but these are less of a feature, and the tumor is more ''solid''. | ||
**[[Calcification]] is uncommon or even rare | **[[Calcification]] is uncommon or even rare. | ||
**''Less'' locally aggressive | **They are ''Less'' locally aggressive. | ||
**''Lower'' rate of recurrence | **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''' | ||
**15%, but shares imaging features and prognosis similar to ''adamantinomatous'' | **15%, but shares imaging features and prognosis similar to ''adamantinomatous''. | ||
==References== | ==References== |
Revision as of 19:15, 24 August 2015
Craniopharyngioma Microchapters |
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Craniopharyngioma classification On the Web |
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Risk calculators and risk factors for Craniopharyngioma classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
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.
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.[1][2]
- Adamantinomatous
- This type is seen predominantly in children.
- 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".
- "Wet keratin nodules" are a characteristic histological feature.
- Calcification is usually present: 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.
- 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.
- 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
- ↑ Histologic classification of Craniopharyngioma. Dr Dylan Kurda and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/craniopharyngioma
- ↑ Classification of Craniopharyngioma. Cancer gov. http://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq#link/_40_toc