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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'''Craniopharyngioma''' is a benign [[neuropathology tumour]].  
{{ Infobox diagnosis
| Name      = Adamantinomatous craniopharyngioma
| Image      = Adamantinomatous_craniopharyngioma_-_very_low_mag.jpg
| Width      =
| Caption    = Adamantinomatous craniopharyngioma. [[HPS stain]].
| Synonyms  =
| Micro      = well-circumscribed (or pseudoinvasive border), multicystic, small-to-medium sized cells with moderate amount of basophilic cytoplasm, bland nuclei (with occ. small nucleoli), "wet" keratin (nests of whorled keratin), calcifications
| Subtypes  =
| LMDDx      =
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      = cystic mass filled with motor oil-like fluid
| Grossing  =
| Site      = [[sella turcica]]
| Assdx      =
| Syndromes  =
| Clinicalhx = adults & children
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  =
| Rads      = classically calcified
| Endoscopy  =
| Prognosis  = benign
| Other      =
| ClinDDx    = other sella turcica lesions
| Tx        =
}}
{{ Infobox diagnosis
| Name      = Papillary craniopharyngioma
| Image      = Papillary craniopharyngioma - high mag.jpg
| Width      =
| Caption    = Papillary craniopharyngioma. [[HPS stain]].
| Synonyms  =
| Micro      = non-keratinized squamous epithelium (without [[nuclear atypia]]), fibrovascular cores (required for ''papillary'')
| Subtypes  =
| LMDDx      =
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = sella turcica
| Assdx      =
| Syndromes  =
| Clinicalhx = adults
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = benign
| Other      =
| ClinDDx    = other sella turcica lesions
| Tx        =
}}
'''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.


Comes in two flavours:<ref name=pmid17425791/>
Subtypes:<ref name=pmid17425791/>
*Adamantinomatous type.
*Adamantinomatous type.
**Adults and children. 
*Squamous papillary type.
*Squamous papillary type.
**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>
 
**Usually solid.
===Adamantinomatous===
*Adults and children.
*Typically contain mutations in CTNNB1 (the gene that encodes β-catenin).<ref name=pmid25355426>{{Cite journal  | last1 = Preda | first1 = V. | last2 = Larkin | first2 = SJ. | last3 = Karavitaki | first3 = N. | last4 = Ansorge | first4 = O. | last5 = Grossman | first5 = AB. | title = The Wnt Signalling Cascade and the Adherens Junction Complex in Craniopharyngioma Tumorigenesis. | journal = Endocr Pathol | volume =  | issue =  | pages =  | month = Oct | year = 2014 | doi = 10.1007/s12022-014-9341-8 | PMID = 25355426 }}</ref>
**Usually intranuclear β-catenin [[immunostain|immunohistochemical]] positivity.
 
===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>
* 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.
 
==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.
====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.
Line 29: Line 120:
*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]]).
Often contain mutations in CTNNB1,  encoding β-catenin<ref name=pmid25355426>{{Cite journal  | last1 = Preda | first1 = V. | last2 = Larkin | first2 = SJ. | last3 = Karavitaki | first3 = N. | last4 = Ansorge | first4 = O. | last5 = Grossman | first5 = AB. | title = The Wnt Signalling Cascade and the Adherens Junction Complex in Craniopharyngioma Tumorigenesis. | journal = Endocr Pathol | volume =  | issue =  | pages =  | month = Oct | year = 2014 | doi = 10.1007/s12022-014-9341-8 | PMID = 25355426 }}</ref> resulting in intranuclear β-catenin immunohistochemical positivity.
 
====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)
Image:Adamantinomatous_craniopharyngioma_-_intermed_mag.jpg | Adamantinomatous craniopharyngioma - intermed. mag. (WC/Nephron)
Image:Adamantinomatous_craniopharyngioma_-_intermed_mag.jpg | Adamantinomatous craniopharyngioma - intermed. mag. (WC/Nephron)
<!-- Image:Adamantinomatous_craniopharyngioma_-_high_mag.jpg  | Adamantinomatous craniopharyngioma - high mag. (WC/Nephron) -->
Image:Adamantinomatous_craniopharyngioma_-_very_high_mag.jpg | Adamantinomatous craniopharyngioma - very high mag. (WC/Nephron)
Image:Adamantinomatous_craniopharyngioma_-_very_high_mag.jpg | Adamantinomatous craniopharyngioma - very high mag. (WC/Nephron)
Image:CNS Craniopharyngeoma Adamantinomatous LP2 PA.JPG|Adamantinomatous Craniopharyngeoma - low power (SKB)
Image:CNS Craniopharyngeoma LP PA.JPG|Adamantinomatous Craniopharyngeoma - low power (SKB)
Image:CNS Craniopharyngeoma MP PA.JPG|Adamantinomatous Craniopharyngeoma - low power (SKB)
Image:CNS Craniopharyngeoma AdamantomatousType MP CTR.jpg|Adamantinomatous Craniopharyngeoma - medium power (SKB)
Image:CNS Craniopharyngeoma AdamantomatousType MP2 CTR.jpg|Adamantinomatous Craniopharyngeoma - medium power (SKB)
Image:CNS Craniopharyngeoma AdamantomatousType MP3 CTR.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: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)
</gallery>
</gallery>


Line 47: Line 150:
*+/-Goblet cell-like formations (rare).
*+/-Goblet cell-like formations (rare).


====Image====
====Images====
<gallery>
<gallery>
Image:Papillary_craniopharyngioma_-_intermed_mag.jpg |Papillary craniopharyngioma - intermed. mag. (WC/Nephron)
Image: Papillary craniopharyngioma - intermed mag.jpg | Papillary craniopharyngioma - intermed. mag. (WC/Nephron)
Image:Papillary_craniopharyngioma_-_very_high_mag.jpg |Papillary craniopharyngioma - very high 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==
Line 62: Line 183:
{{Reflist|2}}
{{Reflist|2}}


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

Latest revision as of 11:56, 11 October 2019

Craniopharyngioma is a benign epithelial neuropathology tumour.

Adamantinomatous craniopharyngioma
Diagnosis in short

Adamantinomatous craniopharyngioma. HPS stain.

LM well-circumscribed (or pseudoinvasive border), multicystic, small-to-medium sized cells with moderate amount of basophilic cytoplasm, bland nuclei (with occ. small nucleoli), "wet" keratin (nests of whorled keratin), calcifications
Gross cystic mass filled with motor oil-like fluid
Site sella turcica

Clinical history adults & children
Radiology classically calcified
Prognosis benign
Clin. DDx other sella turcica lesions
Papillary craniopharyngioma
Diagnosis in short

Papillary craniopharyngioma. HPS stain.

LM non-keratinized squamous epithelium (without nuclear atypia), fibrovascular cores (required for papillary)
Site sella turcica

Clinical history adults
Prognosis benign
Clin. DDx other sella turcica lesions

It is subdivided into papillary craniopharyngioma and adamantinomatous craniopharyngioma.

General

  • Develop from remains of Rathke's pouch or squamous epithelial cell rests.[1]
  • corresponds histologically to WHO grade I.

Subtypes:[1]

  • Adamantinomatous type.
  • Squamous papillary type.

Adamantinomatous

  • Adults and children.
  • Typically contain mutations in CTNNB1 (the gene that encodes β-catenin).[2]

Papillary

  • Adults individuals.[3]
  • Typically contain BRAF V600E mutations.[4]
  • 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:[1]

  • Calcifications (adamantinous type).
  • Contrast enhancing.
  • Cystic portions.

Gross

  • Cystic mass filled with motor oil-like fluid.[5]
    • May not be seen in the papillary variant of craniopharyngioma.
  • Calcified - adamantinomatous type only.
  • Solid & cystic.

Images

Microscopic

Adamantinomatous

Features (adamantinomatous):[6]

  • Trabecular squamous epithelium bordered by palisaded columnar epithelum.
  • Lobules with loosely distributed epithelia ("stellate reticulum").
  • Well-circumscribed (or pseudoinvasive border).
  • Multicystic.
  • Small-to-medium sized cells with moderate amount of basophilic cytoplasm.
  • Bland nuclei (with occ. small nucleoli).
  • "Wet" keratin - nests of whorled keratin.
  • Calcifications (non-psammomatous).

Images

Papillary

Features (papillary):[7]

  • Non-keratinized squamous epithelium (without nuclear atypia).
  • Fibrovascular cores (required for papillary).

Notes:

  • +/-Cilia (rare).
  • +/-Goblet cell-like formations (rare).

Images

www:

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.[11]


See also

References

  1. 1.0 1.1 1.2 Garnett, MR.; Puget, S.; Grill, J.; Sainte-Rose, C. (2007). "Craniopharyngioma.". Orphanet J Rare Dis 2: 18. doi:10.1186/1750-1172-2-18. PMID 17425791.
  2. Preda, V.; Larkin, SJ.; Karavitaki, N.; Ansorge, O.; Grossman, AB. (Oct 2014). "The Wnt Signalling Cascade and the Adherens Junction Complex in Craniopharyngioma Tumorigenesis.". Endocr Pathol. doi:10.1007/s12022-014-9341-8. PMID 25355426.
  3. Giangaspero, F.; Burger, PC.; Osborne, DR.; Stein, RB. (Jan 1984). "Suprasellar papillary squamous epithelioma ("papillary craniopharyngioma").". Am J Surg Pathol 8 (1): 57-64. PMID 6696166.
  4. Brastianos, PK.; Taylor-Weiner, A.; Manley, PE.; Jones, RT.; Dias-Santagata, D.; Thorner, AR.; Lawrence, MS.; Rodriguez, FJ. et al. (Feb 2014). "Exome sequencing identifies BRAF mutations in papillary craniopharyngiomas.". Nat Genet 46 (2): 161-5. doi:10.1038/ng.2868. PMID 24413733.
  5. Fernandez-Miranda, JC.; Gardner, PA.; Snyderman, CH.; Devaney, KO.; Strojan, P.; Suárez, C.; Genden, EM.; Rinaldo, A. et al. (Jul 2012). "Craniopharyngioma: a pathologic, clinical, and surgical review.". Head Neck 34 (7): 1036-44. doi:10.1002/hed.21771. PMID 21584897.
  6. Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 184. ISBN 978-0470519035.
  7. Perry, Arie; Brat, Daniel J. (2010). Practical Surgical Neuropathology: A Diagnostic Approach: A Volume in the Pattern Recognition series (1st ed.). Churchill Livingstone. pp. 406. ISBN 978-0443069826.
  8. URL: http://library.med.utah.edu/WebPath/jpeg4/ENDO115.jpg. Accessed on: 6 December 2010.
  9. URL:[1]. Accessed on: 21 March 2015.
  10. URL:http://neuropathology-web.org/chapter7/chapter7dMiscellaneous.html]. Accessed on: 21 March 2015.
  11. Pascual, JM.; Prieto, R.; Rosdolsky, M.; Hofecker, V.; Strauss, S.; Winter, E.; Ulrich, W. (Sep 2019). "Joseph Engel (1816-1899), author of a meaningful dissertation on tumors of the pituitary infundibulum: his report on the oldest preserved whole craniopharyngioma specimen.". Virchows Arch. doi:10.1007/s00428-019-02664-z. PMID 31511968.