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Difference between revisions of "Endolymphatic sac tumour"
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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 = [[choroid plexus papilloma]], metastatic [[adenocarcinoma]] | |||
| 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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*Locally aggressive.<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 }}</ref> | *Locally aggressive.<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 }}</ref> | ||
*Associated with [[von Hippel-Lindau disease]]. | *Associated with [[von Hippel-Lindau disease]]. | ||
*May be sporadic.<ref name=pmid24966979/> | |||
Clinical - common:<ref name=pmid24436884>{{Cite journal | last1 = Friedman | first1 = RA. | last2 = Hoa | first2 = M. | last3 = Brackmann | first3 = DE. | title = Surgical management of endolymphatic sac tumors. | journal = J Neurol Surg B Skull Base | volume = 74 | issue = 1 | pages = 12-9 | month = Feb | year = 2013 | doi = 10.1055/s-0032-1329622 | PMID = 24436884 }}</ref> | Clinical - common:<ref name=pmid24436884>{{Cite journal | last1 = Friedman | first1 = RA. | last2 = Hoa | first2 = M. | last3 = Brackmann | first3 = DE. | title = Surgical management of endolymphatic sac tumors. | journal = J Neurol Surg B Skull Base | volume = 74 | issue = 1 | pages = 12-9 | month = Feb | year = 2013 | doi = 10.1055/s-0032-1329622 | PMID = 24436884 }}</ref> | ||
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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== | ||
*CD31 +ve - | *CK7 +ve. | ||
*S-100 +ve (focally). | |||
*CD31 +ve - marks the outline of the papillary structures.<ref name=pmid24966979/> | |||
*Kir 7.1 -ve - discriminates tumour from plexus papilloma.<ref name=pmid22706862>{{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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==References== | ==References== | ||
{{Reflist| | {{Reflist|2}} | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Head and neck pathology]] | |||
[[Category:Neuropathology]] | |||
[[Category:Papillary tumour]] | |||