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]].


==General==
==General==
*Rare.
*Rare.
*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]].
*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>
*Hearing loss (sensorineural).
*Tinnitus.
*Dizziness.
Treatment:
*Surgical excision.<ref name=pmid24436884/>


==Microscopic==
==Microscopic==
Features:<ref name=pmid24966979/>
*Cystic spaces.
*Papillary structures.
*Low grade cytomorphology (lack [[nuclear pleomorphism]]).
DDx:
*[[Choroid plexus papilloma]] - when infiltrating cerebellopontine angle via petrous bone.
*[[Metastatic]] [[adenocarcinoma]].


===Images===
===Images===
www:
*[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>
<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==
*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==
Line 14: Line 79:


==References==
==References==
{{Reflist|1}}
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Head and neck pathology]]
[[Category:Neuropathology]]
[[Category:Papillary tumour]]

Latest revision as of 08:57, 29 April 2015

Endolymphatic sac tumour
Diagnosis in short

Endolymphatic sac tumor H&E stain.
LM Papillary epithelial structures.
LM DDx choroid plexus papilloma, metastatic adenocarcinoma
IHC CK7 +ve
Site usually inner ear
Symptoms tinnitus, dizziness
Prevalence very rare
Prognosis locally agressive

Endolymphatic sac tumour, abbreviated ELST, is a very rare tumour associated with von Hippel-Lindau disease.

General

Clinical - common:[2]

  • Hearing loss (sensorineural).
  • Tinnitus.
  • Dizziness.

Treatment:

  • Surgical excision.[2]

Microscopic

Features:[1]

DDx:

Images

www:

  • ELST low power, HE (WC/Soca1zim)

  • ELST high magnification, HE (WC/Soca1zim)

  • ELST very high magnification, HE (WC/jensflorian)

  • Intraoperative frozen section, ELST, HE (WC/jensflorian)

IHC

  • CK7 +ve.
  • S-100 +ve (focally).
  • CD31 +ve - marks the outline of the papillary structures.[1]
  • Kir 7.1 -ve - discriminates tumour from plexus papilloma.[4]

See also

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 Künzel, J.; Agaimy, A.; Hornung, J.; Lell, M.; Ganslandt, O.; Semrau, S.; Zenk, J. (2014). "Sporadic endolymphatic sac tumor--a diagnostic and therapeutic challenge.". Int J Clin Exp Pathol 7 (5): 2641-6. PMID 24966979. Cite error: Invalid <ref> tag; name "pmid24966979" defined multiple times with different content
  2. ↑ 2.0 2.1 Friedman, RA.; Hoa, M.; Brackmann, DE. (Feb 2013). "Surgical management of endolymphatic sac tumors.". J Neurol Surg B Skull Base 74 (1): 12-9. doi:10.1055/s-0032-1329622. PMID 24436884.
  3. ↑ Yang, X.; Liu, XS.; Fang, Y.; Zhang, XH.; Zhang, YK. (2014). "Endolymphatic sac tumor with von Hippel-Lindau disease: report of a case with atypical pathology of endolymphatic sac tumor.". Int J Clin Exp Pathol 7 (5): 2609-14. PMID 24966975.
  4. ↑ Schittenhelm, J.; Roser, F.; Tatagiba, M.; Beschorner, R. (Jul 2012). "Diagnostic value of EAAT-1 and Kir7.1 for distinguishing endolymphatic sac tumors from choroid plexus tumors.". Am J Clin Pathol 138 (1): 85-9. doi:10.1309/AJCPPRKNNL09JTLP. PMID 22706862.
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