Endolymphatic sac tumour

A.Prof Frank Gaillard et al.

Endolymphatic sac tumours (ELST) are very rare, locally invasive tumours of endolymphatic sac. Early detection of these tumours is critical, because early surgical intervention may prevent further hearing loss. Endolymphatic sac tumours do not metastasize but are highly locally aggressive. 

Typically endolymphatic sac tumours are encountered in young individuals, with a mean age at onset is 22 years 2.

Endolymphatic sac tumours typically present with the following symptoms and signs:

  • hearing loss: 95 %, acute in 43% and stepwise in the second half
  • tinnitus: 92%
  • vertigo or disequilibrium: 62%
  • aural fullness: 29%
  • facial paresis: 8%

These tumours are composed of two histological types:

  • mixed type: generally confined
  • papillary adenomatous type: more aggressive and often locally invades the temporal bone

Endolymphatic tumours are most often associated with von Hippel-Lindau disease (vHL) and are detected in 11-16% patients with vHL 2. That having been said, in almost 60% of patients with vHL and vestibulocochlear symptoms, there is no evidence on imaging of an endolymphatic sac tumour 2.  

When these tumours are present in the setting of vHL, then 30% of tumours are bilateral 2.

Always arise from and involve the endolymphatic sac or duct.

When visible, it gives a picture of bone erosion and the "moth-eaten" petrous bone. Endolymphatic sac tumours commonly enhance intensely on CT.

Signal characteristics include:

  • T1: may show high-intensity
  • T1 C+ (Gd): typically show enhancement in the non-cystic component of the tumour
  • T2: often of heterogeneous signal

It was first described in 1989 by Heffner et al. Earlier than this they were probably misdiagnosed as choroid plexus tumours, adenomas, and adenocarcinomas of posterior fossa or cerebellopontine angle. 

Possible imaging differential considerations include

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Article information

rID: 1286
Section: Pathology
Tags: cases, refs
Synonyms or Alternate Spellings:
  • Endolymphatic sac tumor
  • Endolymphatic sac tumour (ELST)
  • Papillary cystadenomatous tumour or temporal bone
  • Papillary adenomatous tumour or temporal bone

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