Craniopharyngioma pathophysiology: Difference between revisions

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{{Craniopharyngioma}}
{{Craniopharyngioma}}
{{CMG}}
{{CMG}};{{AE}}{{Marjan}}
 
==Overview==
Craniopharyngiomas are [[Epithelial|epithelial tumors]] that usually arise in the [[pituitary stalk]] adjacent to the [[optic chiasm]]. On [[gross pathology]], craniopharyngiomas are [[Cystic|cystic or partially cystic]] with solid areas.The [[cyst]] is filled with a [[Turbidity|turbid]], [[Protein|proteinaceous material]] of brownish-yellow color that glitters and sparkles because of a high content of floating [[Cholesterol|cholesterol crystals]]. On [[microscopic]] [[histopathology]], it is categorized into two subtypes: Adamantinomatous and papillary.


==Pathophysiology==
==Pathophysiology==
On gross pathology, craniopharyngiomas are cystic or partially cystic with solid areas. Based on [[histology]], it is categorized into two subtypes: adamantinomatous and papillary. On microscopic histopathological analysis, the [[cysts]] are seen to be lined by [[stratified squamous epithelium]] and [[keratin]] pearls may be present in the adamantinomatous type. It is frequently accompanied by calcium deposition. The papillary type rarely calcifies, and has no keratin pearls.
*Craniopharyngioma is a [[Glial|nonglial]] [[Brain tumor|intracranial tumor]] derived from a [[Congenital disorder|malformation]] of [[Embryonic|embryonic tissue]]. <ref name="pmid1260697">{{cite journal |vauthors=Petito CK, DeGirolami U, Earle KM |title=Craniopharyngiomas: a clinical and pathological review |journal=Cancer |volume=37 |issue=4 |pages=1944–52 |date=April 1976 |pmid=1260697 |doi= |url=}}</ref>
*There are differing [[hypotheses]] on its [[Embryonic|embryonic origin]].
*[[Pathogenesis]] of craniopharyngioma is not completely understood.  
*Some authorities advocate that it originates from [[Ectodermal dysplasia|ectodermal]] [[Ectoderm|remnants]] of [[Rathke's pouch]]. <ref name="pmid6818504">{{cite journal |vauthors=Rush JA, Younge BR, Campbell RJ, MacCarty CS |title=Optic glioma. Long-term follow-up of 85 histopathologically verified cases |journal=Ophthalmology |volume=89 |issue=11 |pages=1213–9 |date=November 1982 |pmid=6818504 |doi= |url=}}</ref>
*Few [[Pathology|pathologists]] believe that it originates from residual [[Embryo|embryonal epithelium]] of the [[Anterior pituitary|anterior pituitary gland]] and of the [[Infundibulum|anterior infundibulum]].  
 
===Embryonic theory===
*According to this theory, craniopharyniomas arise from [[Neoplastic|neoplastic transformation]] of [[Squamous epithelium|embryonic squamous]] [[Cell (biology)|cell nests]] of [[Rathke's pouch]] with the [[stomodeum]].<ref name="pmid9761047">{{cite journal |vauthors=Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM |title=The descriptive epidemiology of craniopharyngioma |journal=J. Neurosurg. |volume=89 |issue=4 |pages=547–51 |date=October 1998 |pmid=9761047 |doi=10.3171/jns.1998.89.4.0547 |url=}}</ref>
*Cell remnants of the [[Craniopharyngeal canal|craniopharyngeal duct]] are spread through the [[intrasellar]] and [[Sella turcica|suprasellar region]].
*The rotation of the [[Anterior pituitary|adenohypophysis]] is caused by different rates of [[Cellular|cellular multiplication]].
*Different rates of [[Cellular migration|cellular multiplication]] results in a [[Cell (biology)|spread of cells]] of the [[Craniopharyngeal canal|craniopharyngeal]] duct to the [[Sella turcica|suprasellar region]].
*The rare location at the [[Cerebellum|cerebellopontine angle]] could fit with this [[hypothesis]].


===Metaplastic theory===
*Papillary craniopharyngiomas are the result of [[metaplasia]] of the [[Pituitary gland|adenohypophyseal]] cells in the [[anterior pituitary]].
*This [[Theory (science)|theory]] is supported by the presence of [[Metaplasticity|metaplastic]] [[Gland|nests in the gland]].<ref name="pmid6818504">{{cite journal |vauthors=Rush JA, Younge BR, Campbell RJ, MacCarty CS |title=Optic glioma. Long-term follow-up of 85 histopathologically verified cases |journal=Ophthalmology |volume=89 |issue=11 |pages=1213–9 |date=November 1982 |pmid=6818504 |doi= |url=}}</ref>
===Gross Pathology===
===Gross Pathology===
On macroscopic examination, craniopharyngiomas are ''cystic'' or ''partially cystic with solid areas''.
*On [[Macroscopic|macroscopic examination]], craniopharyngiomas are [[Cystic|cystic or partially cystic with solid areas]].<ref name="pmid7885544">{{cite journal |vauthors=Weiner HL, Wisoff JH, Rosenberg ME, Kupersmith MJ, Cohen H, Zagzag D, Shiminski-Maher T, Flamm ES, Epstein FJ, Miller DC |title=Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome |journal=Neurosurgery |volume=35 |issue=6 |pages=1001–10; discussion 1010–1 |date=December 1994 |pmid=7885544 |doi= |url=}}</ref>
*Craniopharyngiomas can arise anywhere along the [[craniopharyngeal canal]].
*It occur most often in [[Sella turcica|sellar]] or [[Sella turcica|suprasellar region]] but can have [[Anterior|anterior extension]] to the [[Cistern|prechiasmatic cistern]] and [[Paranasal sinus|subfrontal spaces]].<ref name="pmid7885544">{{cite journal |vauthors=Weiner HL, Wisoff JH, Rosenberg ME, Kupersmith MJ, Cohen H, Zagzag D, Shiminski-Maher T, Flamm ES, Epstein FJ, Miller DC |title=Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome |journal=Neurosurgery |volume=35 |issue=6 |pages=1001–10; discussion 1010–1 |date=December 1994 |pmid=7885544 |doi= |url=}}</ref> 
*[[Posterior|Posterior extension]] into the [[Pontine|prepontine]] , [[third ventricle]], [[posterior fossa]], and [[foramen magnum]]; and [[External rotation|laterally]] toward the [[Temporal|subtemporal spaces]].
*Rare locations of craniopharyngiomas are; the [[nasopharynx]] , [[Paranasal sinus|paranasal area]] , [[sphenoid bone]], [[ethmoid sinus]], [[Chiasmatic cistern|intrachiasmatic area]], [[temporal lobe]].
*Craniopharyngiomas are supplied with [[blood]] coming from the [[Cerebral circulation|anterior cerebral circulation]].<ref name="pmid10690718">{{cite journal |vauthors=Duff J, Meyer FB, Ilstrup DM, Laws ER, Schleck CD, Scheithauer BW |title=Long-term outcomes for surgically resected craniopharyngiomas |journal=Neurosurgery |volume=46 |issue=2 |pages=291–302; discussion 302–5 |date=February 2000 |pmid=10690718 |doi= |url=}}</ref>
*Several [[Cytokines|inflammatory cytokines]] have been shown to be elevated in the craniopharyngioma [[Cyst|cyst fluid]] compared with [[CSF|cerbrospinal fluid]] ([[CSF|CSF)]].<ref name="pmid17630614">{{cite journal |vauthors=Garrè ML, Cama A |title=Craniopharyngioma: modern concepts in pathogenesis and treatment |journal=Curr. Opin. Pediatr. |volume=19 |issue=4 |pages=471–9 |date=August 2007 |pmid=17630614 |doi=10.1097/MOP.0b013e3282495a22 |url=}}</ref>
*[[Tumor]] [[adhesion]] is the result of [[Inflammation|local inflammation]].  


===Microscopic pathology===
===Microscopic pathology===
The [[histologic]] pattern consists of nesting of [[squamous]] [[epithelium]] bordered by radially arranged cells. It is frequently accompanied by calcium deposition and have a microscopic [[papillary]] architecture.
*The [[histologic]] pattern consists of [[Nestin (protein)|nesting]] of [[squamous]] [[epithelium]] bordered by [[Cells|radially arranged cells]].  
Two distinct types are recognized:<ref name="pmid12466115">{{cite journal |author=Sekine S, Shibata T, Kokubu A, ''et al'' |title=Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations |journal=Am. J. Pathol. |volume=161 |issue=6 |pages=1997–2001 |year=2002 |month=December |pmid=12466115 |pmc=1850925 |doi= |url=http://ajp.amjpathol.org/cgi/pmidlookup?view=long&pmid=12466115}}</ref><ref name="pmid15569047">{{cite journal |author=Sekine S, Takata T, Shibata T, ''et al'' |title=Expression of enamel proteins and LEF1 in adamantinomatous craniopharyngioma: evidence for its odontogenic epithelial differentiation |journal=Histopathology |volume=45 |issue=6 |pages=573–9 |year=2004 |month=December |pmid=15569047 |doi=10.1111/j.1365-2559.2004.02029.x |url=http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0309-0167&date=2004&volume=45&issue=6&spage=573}}</ref>
*[[Tumor]] is frequently accompanied by [[Calcium|calcium deposition]] and have a [[microscopic]] [[papillary]] architecture.<ref name="pmid9761047">{{cite journal |vauthors=Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM |title=The descriptive epidemiology of craniopharyngioma |journal=J. Neurosurg. |volume=89 |issue=4 |pages=547–51 |date=October 1998 |pmid=9761047 |doi=10.3171/jns.1998.89.4.0547 |url=}}</ref>
*Adamantinomatous craniopharyngioma
*Two distinct types are recognized:<ref name="pmid12466115">{{cite journal |author=Sekine S, Shibata T, Kokubu A, ''et al'' |title=Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations |journal=Am. J. Pathol. |volume=161 |issue=6 |pages=1997–2001 |year=2002 |month=December |pmid=12466115 |pmc=1850925 |doi= |url=http://ajp.amjpathol.org/cgi/pmidlookup?view=long&pmid=12466115}}</ref><ref name="pmid15569047">{{cite journal |author=Sekine S, Takata T, Shibata T, ''et al'' |title=Expression of enamel proteins and LEF1 in adamantinomatous craniopharyngioma: evidence for its odontogenic epithelial differentiation |journal=Histopathology |volume=45 |issue=6 |pages=573–9 |year=2004 |month=December |pmid=15569047 |doi=10.1111/j.1365-2559.2004.02029.x |url=http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0309-0167&date=2004&volume=45&issue=6&spage=573}}</ref>  
*Papillary craniopharyngioma.
#Adamantinomatous craniopharyngioma
 
#Papillary craniopharyngioma
''In the adamantinomatous type, calcifications are visible on neuroimaging and are helpful in diagnosis''. ''The papillary type rarely calcifies''. ''On light microscopy, the cysts are seen to be lined by [[stratified squamous epithelium]]''. ''[[Keratin]] pearls may also be seen''. ''The [[cysts]] are usually filled with a yellow, viscous fluid which is rich in [[cholesterol]] crystals''. ''In addition to a long list of possible symptoms, the most common presentation include: headaches, growth failure, and [[bitemporal hemianopsia]]''.
*Shown below is a micrograph showing the characteristic features of an '''adamantinomatous''' craniopharyngioma - cystic spaces, calcifications, and "wet [[keratin]]" pearls on hematoxylin phloxine saffron (HPS) stain.


*In the adamantinomatous type, [[Calcification|calcifications]] are visible on [[neuroimaging]] and are helpful in [[diagnosis]].
*The papillary type rarely calcifies. On [[Microscopy|light microscopy]], the [[Cyst|cysts]] are seen to be lined by [[Squamous epithelium|stratified squamous epithelium]].
*[[Keratin|Keratin pearls]] may also be seen.
*The [[Cyst|cysts]] are usually filled with a yellow, [[Viscous|viscous fluid]] which is rich in [[Cholesterol|cholesterol crystals]].
*[[Cells|Islands of densely packed cells]] merge with loosely cohesive aggregates of squamous cells known as [[stellate reticulum]].
*Nodules of “wet keratin” representing remnants of pale nuclei embedded within an [[Eosinophilic|eosinophilic keratinous mass]] may be found.
*[[Cystic|Cystic cavities]] containing [[Squamous epithelium|squamous]] debris are lined by [[Epithelium|flattened epithelium]].
*[[Granulomatous|Granulomatous inflammation]] associated with [[Cholesterol|cholesterol clefts]] and [[Macrophage|giant cells]] may be detectable, but this is more typical for [[Xanthogranulomatous inflammation|xanthogranuloma]].<ref name="pmid6818504">{{cite journal |vauthors=Rush JA, Younge BR, Campbell RJ, MacCarty CS |title=Optic glioma. Long-term follow-up of 85 histopathologically verified cases |journal=Ophthalmology |volume=89 |issue=11 |pages=1213–9 |date=November 1982 |pmid=6818504 |doi= |url=}}</ref>
*[[Pilocytic astrocytoma|Piloid gliosis]] with abundant [[Rosenthal fiber|Rosenthal fibers]] is often seen at the [[Mass|infiltrative interface]] of the [[tumor]] and should not be mistaken for [[pilocytic astrocytoma]].
*[[Malignant transformation]] of craniopharyngioma appears to be very rare.


[[Image:800px-Adamantinomatous_craniopharyngioma_-_intermed_mag.jpg‎|200px]]
==Genetics==
*Shown below is a Micrograph showing a '''papillary''' craniopharyngioma on HPS stain.
*Multiple [[Chromosome abnormality|chromosomal abnormalities]] have been reported by classic [[Cytogenetics|cytogenetic analysis]].
 
*Most [[Tumor|tumors]] had abnormalities involving [[Chromosome|chromosomes]] 2 and 12.
 
*More than 70% of adamantinomatous craniopharyngiomas harbor a [[mutation]] of the [[Β-catenin|β-catenin gene]].
[[Image:800px-Papillary_craniopharyngioma_-_high_mag.jpg‎|200px]]
*Most of the [[Mutation|mutations]] affect [[Exon|exon 3]], which encodes the degradation targeting [[Box|box of]] [[Beta-catenin|β-catenin]].
*No mutations have been demonstrated in papillary craniopharyngioma.
*Comparative [[Hybridization|genomic hybridization]] studies on two large series of craniopharyngiomas have failed to show significant [[Chromosome abnormality|chromosomal imbalances]].
*The mutations of genes encoding [[Beta-catenin|β-catenin]] [[Catenin|(CTNNB1 and APC)]] are an exclusive characteristic of adamantinomatous craniopharyngioma.
*Few researchers demonstrated a [[Catenin|causative effect of mutant]] [[Beta-catenin|β-catenin]] in the [[etiology]] of human adamantinomatous craniopharyngioma.


==References==
==References==
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Latest revision as of 15:05, 26 February 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

Overview

Craniopharyngiomas are epithelial tumors that usually arise in the pituitary stalk adjacent to the optic chiasm. On gross pathology, craniopharyngiomas are cystic or partially cystic with solid areas.The cyst is filled with a turbid, proteinaceous material of brownish-yellow color that glitters and sparkles because of a high content of floating cholesterol crystals. On microscopic histopathology, it is categorized into two subtypes: Adamantinomatous and papillary.

Pathophysiology

Embryonic theory

Metaplastic theory

Gross Pathology

Microscopic pathology

  1. Adamantinomatous craniopharyngioma
  2. Papillary craniopharyngioma

Genetics

References

  1. Petito CK, DeGirolami U, Earle KM (April 1976). "Craniopharyngiomas: a clinical and pathological review". Cancer. 37 (4): 1944–52. PMID 1260697.
  2. 2.0 2.1 2.2 Rush JA, Younge BR, Campbell RJ, MacCarty CS (November 1982). "Optic glioma. Long-term follow-up of 85 histopathologically verified cases". Ophthalmology. 89 (11): 1213–9. PMID 6818504.
  3. 3.0 3.1 Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM (October 1998). "The descriptive epidemiology of craniopharyngioma". J. Neurosurg. 89 (4): 547–51. doi:10.3171/jns.1998.89.4.0547. PMID 9761047.
  4. 4.0 4.1 Weiner HL, Wisoff JH, Rosenberg ME, Kupersmith MJ, Cohen H, Zagzag D, Shiminski-Maher T, Flamm ES, Epstein FJ, Miller DC (December 1994). "Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome". Neurosurgery. 35 (6): 1001–10, discussion 1010–1. PMID 7885544.
  5. Duff J, Meyer FB, Ilstrup DM, Laws ER, Schleck CD, Scheithauer BW (February 2000). "Long-term outcomes for surgically resected craniopharyngiomas". Neurosurgery. 46 (2): 291–302, discussion 302–5. PMID 10690718.
  6. Garrè ML, Cama A (August 2007). "Craniopharyngioma: modern concepts in pathogenesis and treatment". Curr. Opin. Pediatr. 19 (4): 471–9. doi:10.1097/MOP.0b013e3282495a22. PMID 17630614.
  7. Sekine S, Shibata T, Kokubu A; et al. (2002). "Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations". Am. J. Pathol. 161 (6): 1997–2001. PMC 1850925. PMID 12466115. Unknown parameter |month= ignored (help)
  8. Sekine S, Takata T, Shibata T; et al. (2004). "Expression of enamel proteins and LEF1 in adamantinomatous craniopharyngioma: evidence for its odontogenic epithelial differentiation". Histopathology. 45 (6): 573–9. doi:10.1111/j.1365-2559.2004.02029.x. PMID 15569047. Unknown parameter |month= ignored (help)


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