Difference between revisions of "Craniopharyngioma"

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'''Craniopharyngioma''' is a benign [[neuropathology tumour]]. It is subdivided into '''papillary craniopharyngioma''' and '''adamantinomatous craniopharyngioma'''.
{{ 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'''.


==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/>
Radiology:<ref name=pmid17425791/>
*Calcifications (adamantinous type).
*Contrast enhancing.
*Cystic portions.
==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>
**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.
Line 23: 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]]).


====Images====  
====Images====  
Line 29: Line 126:
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>
===Papillary===
===Papillary===
Features (papillary):<ref name=Ref_PSNP406>{{Ref PSNP|406}}</ref>
Features (papillary):<ref name=Ref_PSNP406>{{Ref PSNP|406}}</ref>
Line 40: 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 55: 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

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

Craniopharyngioma is a benign epithelial neuropathology tumour.

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

  • Calcifications in adamantinomatous craniopharyngioma. (WC/Garnett et al.)

  • Autopsy case with papillary craniopharyngioma of the 3rd ventricle. (WC/AFIP)

  • Cholesterol crystals, a typical finding in the cyst fluid, are readily identified by examination under polarized light. (WC/AFIP)

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

  • Adamantinomatous craniopharyngioma - very low mag. (WC/Nephron)

  • Adamantinomatous craniopharyngioma - intermed. mag. (WC/Nephron)

  • Adamantinomatous craniopharyngioma - very high mag. (WC/Nephron)

  • Adamantinomatous Craniopharyngeoma - low power (SKB)

  • Adamantinomatous Craniopharyngeoma - low power (SKB)

  • Adamantinomatous Craniopharyngeoma - low power (SKB)

  • Adamantinomatous Craniopharyngeoma - medium power (SKB)

  • Adamantinomatous Craniopharyngeoma - medium power (SKB)

  • Adamantinomatous Craniopharyngeoma - medium power (SKB)

  • Adamantinomatous Craniopharyngeoma - medium power (SKB)

  • Adamantinomatous Craniopharyngeoma - medium power (SKB)

  • Brain infiltration. "Wet" keratin present in two tumor protrusions. The surrounding CNS shows extensive piloid gliosis. (WC/jensflorian)

  • Adamantinomatous Craniopharyngeoma - Trabecular growth pattern. (WC/jensflorian)

  • Adamantinomatous Craniopharyngeoma - Intraoperative frozen section. (WC/jensflorian)

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

  • Papillary craniopharyngioma - intermed. mag. (WC/Nephron)

  • Papillary craniopharyngioma - high mag. (WC/Nephron)

  • Papillary craniopharyngioma - very high mag. (WC/Nephron)

  • Papillary Craniopharyngeoma - low power (SKB)

  • Papillary Craniopharyngeoma - low power (SKB)

  • Papillary Craniopharyngeoma - low power (SKB)

  • Papillary Craniopharyngeoma - medium power (SKB)

  • Papillary Craniopharyngeoma - medium power (SKB)

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.
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