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| 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== |
*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 | is... |
*corresponds histologically to WHO grade I. |
Subtypes:<ref name=pmid17425791/> |
*Adamantinomatous type. |
*Squamous papillary type. |
===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 ... |
**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 | ... |
* 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 = M... |
*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== |
*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 = ... |
**May not be seen in the papillary variant of craniopharyngioma. |
*Calcified - adamantinomatous type only. |
*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== |
===Adamantinomatous=== |
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). |
*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-[[psammoma bodies|psammomatous]]). |
====Images==== |
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