Difference between revisions of "Endolymphatic sac tumour"

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pictures + infobox
(pictures + infobox)
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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = ELST_high_magnification_HE.jpg
| Width      =
| Caption    = Endolymphatic sac tumor [[H&E stain]].
| Synonyms  =
| Micro      = Papillary epithelial structures.
| Subtypes  =
| LMDDx      = [[Plexus papilloma]]
| Stains    =
| IHC        = CK7 +ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = usually inner ear
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  = Tinnitus, dizziness
| Prevalence = very rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = locally agressive
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Endolymphatic sac tumour''', abbreviated '''ELST''', is a very rare tumour associated with [[von Hippel-Lindau disease]].
'''Endolymphatic sac tumour''', abbreviated '''ELST''', is a very rare tumour associated with [[von Hippel-Lindau disease]].


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*Papillary structures.
*Papillary structures.
*Low grade cytomorphology (lack [[nuclear pleomorphism]]).
*Low grade cytomorphology (lack [[nuclear pleomorphism]]).
DDx:
*Choroid plexus papilloma - when infiltrating cerebellopontine angle via petrous bone
*Metastatic [[adenocarcinoma]]


===Images===
===Images===
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*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4069932/figure/fig02/ ELST (nih.gov)].<ref name=pmid24966975>{{Cite journal  | last1 = Yang | first1 = X. | last2 = Liu | first2 = XS. | last3 = Fang | first3 = Y. | last4 = Zhang | first4 = XH. | last5 = Zhang | first5 = YK. | title = Endolymphatic sac tumor with von Hippel-Lindau disease: report of a case with atypical pathology of endolymphatic sac tumor. | journal = Int J Clin Exp Pathol | volume = 7 | issue = 5 | pages = 2609-14 | month =  | year = 2014 | doi =  | PMID = 24966975 }}</ref>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4069932/figure/fig02/ ELST (nih.gov)].<ref name=pmid24966975>{{Cite journal  | last1 = Yang | first1 = X. | last2 = Liu | first2 = XS. | last3 = Fang | first3 = Y. | last4 = Zhang | first4 = XH. | last5 = Zhang | first5 = YK. | title = Endolymphatic sac tumor with von Hippel-Lindau disease: report of a case with atypical pathology of endolymphatic sac tumor. | journal = Int J Clin Exp Pathol | volume = 7 | issue = 5 | pages = 2609-14 | month =  | year = 2014 | doi =  | PMID = 24966975 }}</ref>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4069893/figure/fig03/ ELST (nih.gov)].<ref name=pmid24966979>{{Cite journal  | last1 = Künzel | first1 = J. | last2 = Agaimy | first2 = A. | last3 = Hornung | first3 = J. | last4 = Lell | first4 = M. | last5 = Ganslandt | first5 = O. | last6 = Semrau | first6 = S. | last7 = Zenk | first7 = J. | title = Sporadic endolymphatic sac tumor--a diagnostic and therapeutic challenge. | journal = Int J Clin Exp Pathol | volume = 7 | issue = 5 | pages = 2641-6 | month =  | year = 2014 | doi =  | PMID = 24966979 | PMC = 4069893}}</ref>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4069893/figure/fig03/ ELST (nih.gov)].<ref name=pmid24966979>{{Cite journal  | last1 = Künzel | first1 = J. | last2 = Agaimy | first2 = A. | last3 = Hornung | first3 = J. | last4 = Lell | first4 = M. | last5 = Ganslandt | first5 = O. | last6 = Semrau | first6 = S. | last7 = Zenk | first7 = J. | title = Sporadic endolymphatic sac tumor--a diagnostic and therapeutic challenge. | journal = Int J Clin Exp Pathol | volume = 7 | issue = 5 | pages = 2641-6 | month =  | year = 2014 | doi =  | PMID = 24966979 | PMC = 4069893}}</ref>
<gallery>
File:Ear_Endolymphatic_Sac_Tumor_H_and_E_Low_power_LDRT.tif | ELST low power, HE (WC/Soca1zim)
File:Ear Endolymphatic Sac Tumor H & E High power LDRT.tif | ELST high magnification, HE (WC/Soca1zim)
File:ELST_high_magnification_HE.jpg | ELST very high magnification, HE (WC/jensflorian)
File:ELST_frozen_section_HE.jpg | Intraoperative frozen section, ELST, HE (WC/jensflorian)
</gallery>


==IHC==
==IHC==
*CK7 +ve
*S-100 +ve (focally)
*CD31 +ve - marks the outline of the papillary structures.<ref name=pmid24966979/>
*CD31 +ve - marks the outline of the papillary structures.<ref name=pmid24966979/>
*Kir 7.1-ve - discriminates tumor from plexus papilloma.<ref>{{Cite journal  | last1 = Schittenhelm | first1 = J. | last2 = Roser | first2 = F. | last3 = Tatagiba | first3 = M. | last4 = Beschorner | first4 = R. | title = Diagnostic value of EAAT-1 and Kir7.1 for distinguishing endolymphatic sac tumors from choroid plexus tumors. | journal = Am J Clin Pathol | volume = 138 | issue = 1 | pages = 85-9 | month = Jul | year = 2012 | doi = 10.1309/AJCPPRKNNL09JTLP | PMID = 22706862 }}</ref>


==See also==
==See also==
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