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The oldest specimen from 1828 is displayed in the pathological-anatomical museum of Vienna.
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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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{{ Infobox diagnosis | {{ Infobox diagnosis | ||
| Name = Adamantinomatous craniopharyngioma | | Name = Adamantinomatous craniopharyngioma | ||
| Image = Adamantinomatous_craniopharyngioma_-_very_low_mag.jpg | | Image = Adamantinomatous_craniopharyngioma_-_very_low_mag.jpg | ||
| Width = | | Width = | ||
| Caption = Adamantinomatous craniopharyngioma. [[HPS stain]]. | | Caption = Adamantinomatous craniopharyngioma. [[HPS stain]]. | ||
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| Tx = | | Tx = | ||
}} | }} | ||
'''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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==General== | ==General== | ||
*Develop from remains of Rathke's pouch or squamous epithelial cell rests.<ref name=pmid17425791>{{Cite journal | last1 = Garnett | first1 = MR. | last2 = Puget | first2 = S. | last3 = Grill | first3 = J. | last4 = Sainte-Rose | first4 = C. | title = Craniopharyngioma. | journal = Orphanet J Rare Dis | volume = 2 | issue = | pages = 18 | month = | year = 2007 | doi = 10.1186/1750-1172-2-18 | PMID = 17425791 }}</ref> | *Develop from remains of Rathke's pouch or squamous epithelial cell rests.<ref name=pmid17425791>{{Cite journal | last1 = Garnett | first1 = MR. | last2 = Puget | first2 = S. | last3 = Grill | first3 = J. | last4 = Sainte-Rose | first4 = C. | title = Craniopharyngioma. | journal = Orphanet J Rare Dis | volume = 2 | issue = | pages = 18 | month = | year = 2007 | doi = 10.1186/1750-1172-2-18 | PMID = 17425791 }}</ref> | ||
*corresponds histologically to WHO grade I. | |||
Subtypes:<ref name=pmid17425791/> | Subtypes:<ref name=pmid17425791/> | ||
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===Papillary=== | ===Papillary=== | ||
*Adults individuals.<ref name=pmid6696166>{{Cite journal | last1 = Giangaspero | first1 = F. | last2 = Burger | first2 = PC. | last3 = Osborne | first3 = DR. | last4 = Stein | first4 = RB. | title = Suprasellar papillary squamous epithelioma ("papillary craniopharyngioma"). | journal = Am J Surg Pathol | volume = 8 | issue = 1 | pages = 57-64 | month = Jan | year = 1984 | doi = | PMID = 6696166 }}</ref> | *Adults individuals.<ref name=pmid6696166>{{Cite journal | last1 = Giangaspero | first1 = F. | last2 = Burger | first2 = PC. | last3 = Osborne | first3 = DR. | last4 = Stein | first4 = RB. | title = Suprasellar papillary squamous epithelioma ("papillary craniopharyngioma"). | journal = Am J Surg Pathol | volume = 8 | issue = 1 | pages = 57-64 | month = Jan | year = 1984 | doi = | PMID = 6696166 }}</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. | ||
====Images==== | |||
<gallery> | |||
File:Craniopharyngioma1.jpg|Calcifications in adamantinomatous craniopharyngioma. (WC/Garnett et al.) | |||
File:Papillary craniopharyngioma.jpg|Autopsy case with papillary craniopharyngioma of the 3rd ventricle. (WC/AFIP) | |||
File:Adamantinomatous craniopharyngioma.jpg|Cholesterol crystals, a typical finding in the cyst fluid, are readily identified by examination under polarized light. (WC/AFIP) | |||
</gallery> | |||
==Microscopic== | ==Microscopic== | ||
===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"). | |||
*Well-circumscribed (or pseudoinvasive border). | *Well-circumscribed (or pseudoinvasive border). | ||
*Multicystic. | *Multicystic. | ||
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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==== | ||
<gallery> | <gallery> | ||
Image:Adamantinomatous_craniopharyngioma_-_very_low_mag.jpg | Adamantinomatous craniopharyngioma - very low mag. (WC/Nephron) | Image:Adamantinomatous_craniopharyngioma_-_very_low_mag.jpg | Adamantinomatous craniopharyngioma - very low mag. (WC/Nephron) | ||
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Image:CNS Craniopharyngeoma Adamantinomatous MP MP PA.JPG|Adamantinomatous Craniopharyngeoma - medium power (SKB) | Image:CNS Craniopharyngeoma Adamantinomatous MP MP PA.JPG|Adamantinomatous Craniopharyngeoma - medium power (SKB) | ||
Image:CNS Craniopharyngeoma Adamantinomatous 2 MP PA.JPG|Adamantinomatous Craniopharyngeoma - medium power (SKB) | Image:CNS Craniopharyngeoma Adamantinomatous 2 MP PA.JPG|Adamantinomatous Craniopharyngeoma - medium power (SKB) | ||
Image:Craniopharyngeoma CNS infiltration.jpg|Brain infiltration. "Wet" keratin present in two tumor protrusions. The surrounding CNS shows extensive piloid gliosis. (WC/jensflorian) | |||
Image:Craniopharyngioma histology.jpg|Adamantinomatous Craniopharyngeoma - Trabecular growth pattern. (WC/jensflorian) | |||
Image:Craniopharyngioma_HE_frozen.jpg|Adamantinomatous Craniopharyngeoma - Intraoperative frozen section. (WC/jensflorian) | |||
Image:CNS | |||
Image: | |||
Image: | |||
</gallery> | </gallery> | ||
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*+/-Goblet cell-like formations (rare). | *+/-Goblet cell-like formations (rare). | ||
==== | ====Images==== | ||
<gallery> | <gallery> | ||
Image: Papillary craniopharyngioma - intermed mag.jpg | Papillary craniopharyngioma - intermed. mag. (WC/Nephron) | |||
Image: Papillary craniopharyngioma - high mag.jpg | Papillary craniopharyngioma - high mag. (WC/Nephron) | |||
Image: Papillary craniopharyngioma - very high mag.jpg | Papillary craniopharyngioma - very high mag. (WC/Nephron) | |||
Image:CNS Craniopharyngeoma Papillary LP2 PA.JPG|Papillary Craniopharyngeoma - low power (SKB) | |||
Image:CNS Craniopharyngeoma Papillary LP PA.JPG|Papillary Craniopharyngeoma - low power (SKB) | |||
Image:CNS Craniopharyngeoma Papillary LP PA (2) copy.jpg|Papillary Craniopharyngeoma - low power (SKB) | |||
Image:CNS Craniopharyngeoma Papillary MP PA.JPG|Papillary Craniopharyngeoma - medium power (SKB) | |||
Image:CNS Craniopharyngioma Papillary 3 MP PA.JPG|Papillary Craniopharyngeoma - medium power (SKB) | |||
</gallery> | </gallery> | ||
www: | www: | ||
*[http://library.med.utah.edu/WebPath/jpeg4/ENDO115.jpg Craniopharyngioma (med.utah.edu)].<ref>URL: [http://library.med.utah.edu/WebPath/jpeg4/ENDO115.jpg http://library.med.utah.edu/WebPath/jpeg4/ENDO115.jpg]. Accessed on: 6 December 2010.</ref> | *[http://library.med.utah.edu/WebPath/jpeg4/ENDO115.jpg Craniopharyngioma (med.utah.edu)].<ref>URL: [http://library.med.utah.edu/WebPath/jpeg4/ENDO115.jpg http://library.med.utah.edu/WebPath/jpeg4/ENDO115.jpg]. Accessed on: 6 December 2010.</ref> | ||
*[http://www.cnsatlas.com/cgi-bin/nephrology/preview?ADD=0&LESION_ID=382&BOOK_ID=5&POST=toc Craniopharyngioma at cnsatlas.com].<ref>URL:[http://www.cnsatlas.com/cgi-bin/nephrology/preview?ADD=0&LESION_ID=382&BOOK_ID=5&POST=toc]. Accessed on: 21 March 2015.</ref> | |||
*[http://neuropathology-web.org/chapter7/chapter7dMiscellaneous.html Craniopharyngioma at Neuropathology-web.org]<ref>URL:http://neuropathology-web.org/chapter7/chapter7dMiscellaneous.html]. Accessed on: 21 March 2015.</ref> | |||
===Differential diagnosis=== | |||
*Xanthogranuloma | |||
*Rathke cyst | |||
*Epidermoid | |||
*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]] | |||