Difference between revisions of "Endolymphatic sac tumour"

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| Micro      = Papillary epithelial structures.
| Micro      = Papillary epithelial structures.
| Subtypes  =
| Subtypes  =
| LMDDx      = [[choriod plexus papilloma]]  
| LMDDx      = [[choroid plexus papilloma]], metastatic [[adenocarcinoma]]  
| Stains    =  
| Stains    =  
| IHC        = CK7 +ve
| IHC        = CK7 +ve
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| Clinicalhx =
| Clinicalhx =
| Signs      =  
| Signs      =  
| Symptoms  = Tinnitus, dizziness
| Symptoms  = tinnitus, dizziness
| Prevalence = very rare
| Prevalence = very rare
| Bloodwork  =
| Bloodwork  =
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| Tx        =
| 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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*S-100 +ve (focally).
*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 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>
*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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[[Category:Head and neck pathology]]
[[Category:Head and neck pathology]]
[[Category:Neuropathology]]
[[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:

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.