Endolymphatic sac tumour

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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.


Clinical - common:[2]

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


  • Surgical excision.[2]







  • 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


  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.