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Jensflorian (talk | contribs) (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. | ||
*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 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]] |