Difference between revisions of "Craniopharyngioma"

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The oldest specimen from 1828 is displayed in the pathological-anatomical museum of Vienna.
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(The oldest specimen from 1828 is displayed in the pathological-anatomical museum of Vienna.)
 
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'''Craniopharyngioma''' is a benign [[neuropathology tumour]].  
'''Craniopharyngioma''' is a benign epithelial [[neuropathology tumour]].  


It is subdivided into '''papillary craniopharyngioma''' and '''adamantinomatous craniopharyngioma'''.
It is subdivided into '''papillary craniopharyngioma''' and '''adamantinomatous craniopharyngioma'''.
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* Typically contain BRAF V600E mutations.<ref name=pmid24413733>{{Cite journal  | last1 = Brastianos | first1 = PK. | last2 = Taylor-Weiner | first2 = A. | last3 = Manley | first3 = PE. | last4 = Jones | first4 = RT. | last5 = Dias-Santagata | first5 = D. | last6 = Thorner | first6 = AR. | last7 = Lawrence | first7 = MS. | last8 = Rodriguez | first8 = FJ. | last9 = Bernardo | first9 = LA. | title = Exome sequencing identifies BRAF mutations in papillary craniopharyngiomas. | journal = Nat Genet | volume = 46 | issue = 2 | pages = 161-5 | month = Feb | year = 2014 | doi = 10.1038/ng.2868 | PMID = 24413733 }}</ref>
* Typically contain BRAF V600E mutations.<ref name=pmid24413733>{{Cite journal  | last1 = Brastianos | first1 = PK. | last2 = Taylor-Weiner | first2 = A. | last3 = Manley | first3 = PE. | last4 = Jones | first4 = RT. | last5 = Dias-Santagata | first5 = D. | last6 = Thorner | first6 = AR. | last7 = Lawrence | first7 = MS. | last8 = Rodriguez | first8 = FJ. | last9 = Bernardo | first9 = LA. | title = Exome sequencing identifies BRAF mutations in papillary craniopharyngiomas. | journal = Nat Genet | volume = 46 | issue = 2 | pages = 161-5 | month = Feb | year = 2014 | doi = 10.1038/ng.2868 | PMID = 24413733 }}</ref>
*Usually solid.
*Usually solid.
==Clinical features==
*Usu. located in the suprasellar cistern.
**Rare locations: Cerebellopontine angle, sphenoid sinus, third ventricle.
*Visual problems.
*Endocrine deficiencies.
*Hypothalamic dysfunction (obesity).
*More frequent in asia than in Europe/US.
==Imaging==
Radiology:<ref name=pmid17425791/>
*Calcifications (adamantinous type).
*Contrast enhancing.
*Cystic portions.


==Gross==
==Gross==
*Cystic mass filled with motor oil-like fluid.<ref name=pmid21584897>{{Cite journal  | last1 = Fernandez-Miranda | first1 = JC. | last2 = Gardner | first2 = PA. | last3 = Snyderman | first3 = CH. | last4 = Devaney | first4 = KO. | last5 = Strojan | first5 = P. | last6 = Suárez | first6 = C. | last7 = Genden | first7 = EM. | last8 = Rinaldo | first8 = A. | last9 = Ferlito | first9 = A. | title = Craniopharyngioma: a pathologic, clinical, and surgical review. | journal = Head Neck | volume = 34 | issue = 7 | pages = 1036-44 | month = Jul | year = 2012 | doi = 10.1002/hed.21771 | PMID = 21584897 }}</ref>
*Cystic mass filled with motor oil-like fluid.<ref name=pmid21584897>{{Cite journal  | last1 = Fernandez-Miranda | first1 = JC. | last2 = Gardner | first2 = PA. | last3 = Snyderman | first3 = CH. | last4 = Devaney | first4 = KO. | last5 = Strojan | first5 = P. | last6 = Suárez | first6 = C. | last7 = Genden | first7 = EM. | last8 = Rinaldo | first8 = A. | last9 = Ferlito | first9 = A. | title = Craniopharyngioma: a pathologic, clinical, and surgical review. | journal = Head Neck | volume = 34 | issue = 7 | pages = 1036-44 | month = Jul | year = 2012 | doi = 10.1002/hed.21771 | PMID = 21584897 }}</ref>
**May not be seen in the papillary variant of craniopharyngioma.
**May not be seen in the papillary variant of craniopharyngioma.
Radiology:<ref name=pmid17425791/>
*Calcified - adamantinomatous type only.
*Calcified - adamantinomatous type only.
*Solid & cystic.
*Solid & cystic.
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===Adamantinomatous===
===Adamantinomatous===
Features (adamantinomatous):<ref name=Ref_DCHH184>{{Ref DCHH|184}}</ref>
Features (adamantinomatous):<ref name=Ref_DCHH184>{{Ref DCHH|184}}</ref>
*trabecular squamous epithelium bordered by palisaded columar epithelum.
*Trabecular squamous epithelium bordered by palisaded columnar epithelum.
*Lobules with loosely distributed epithelia ("stellate reticulum").
*Lobules with loosely distributed epithelia ("stellate reticulum").
*Well-circumscribed (or pseudoinvasive border).
*Well-circumscribed (or pseudoinvasive border).
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*Bland nuclei (with occ. small nucleoli).
*Bland nuclei (with occ. small nucleoli).
*"Wet" keratin - nests of whorled keratin.
*"Wet" keratin - nests of whorled keratin.
*Calcifications (non-psammomatous).
*Calcifications (non-[[psammoma bodies|psammomatous]]).


====Images====  
====Images====  
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*Epidermoid
*Epidermoid
*Well-differentiated carcinoma metastasis
*Well-differentiated carcinoma metastasis
==Trivia==
*The oldest specimen from 1828 is displayed in the pathological-anatomical museum of Vienna.<ref>{{Cite journal  | last1 = Pascual | first1 = JM. | last2 = Prieto | first2 = R. | last3 = Rosdolsky | first3 = M. | last4 = Hofecker | first4 = V. | last5 = Strauss | first5 = S. | last6 = Winter | first6 = E. | last7 = Ulrich | first7 = W. | title = Joseph Engel (1816-1899), author of a meaningful dissertation on tumors of the pituitary infundibulum: his report on the oldest preserved whole craniopharyngioma specimen. | journal = Virchows Arch | volume =  | issue =  | pages =  | month = Sep | year = 2019 | doi = 10.1007/s00428-019-02664-z | PMID = 31511968 }}</ref>


==See also==
==See also==
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{{Reflist|2}}
{{Reflist|2}}


[[Category:Neuropathology]]
[[Category:Neuropathology tumours]]
[[Category:Diagnosis]]
[[Category:Papillary tumour]]
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