Endolymphatic sac tumor
Updates to Article Attributes
Endolymphatic sac tumours (ELST) are very rare, locally invasive tumorstumours of endolymphatic sac. Early detection of ELST is very importantcritical, because early surgical intervention may prevent further hearing loss. ELSTs do not metastasise, but are highly locally aggressive.
Epidemiology
Mean age at onset is 22 years.
Clinical presentation
ELSTs 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%
Pathology
Composed of two histological types:
- mixed type: generally confined
- papillary adenomatous type: more aggressive and often locally invades the temporal bone
Associations
Most often associated with von Hippel-Lindau disease (vHL). ELSTs are detected in 11-16% patients with vHL ref. In almost 60% of patients with VHLvHL and vestibulocochlear symptoms, there is no evidence on imaging of an endolymphatic-sac tumor sac tumour ref.
30% of tumours in vHL patients are bilateral.
Radiographic features
CT
When visible, it gives a picture of bone erosion and the "moth-eaten" petrous bone. ELSTs commonly enhance intensely on CT.
MRI
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
heterogenousheterogeneous signal
History and etymology
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.
Differential diagnosis
Other tumours of the cerebellopontine angle and posterior fossa.
-<p><strong>Endolymphatic sac tumours (ELST)</strong> are very rare, locally invasive tumors of <a href="/articles/endolymphatic-sac">endolymphatic sac</a>. Early detection of ELST is very important, because early surgical intervention may prevent further hearing loss. ELSTs do not metastasise, but are highly locally aggressive. </p><h4>Epidemiology</h4><p>Mean age at onset is 22 years.</p><h4>Clinical presentation</h4><p>ELSTs present with the following symptoms and signs:</p><ul>-<li>hearing loss: 95 %, acute in 43% and stepwise in second half</li>- +<p><strong>Endolymphatic sac tumours (ELST)</strong> are very rare, locally invasive tumours of <a href="/articles/endolymphatic-sac">endolymphatic sac</a>. Early detection of ELST is critical, because early surgical intervention may prevent further hearing loss. ELSTs do not metastasise but are highly locally aggressive. </p><h4>Epidemiology</h4><p>Mean age at onset is 22 years.</p><h4>Clinical presentation</h4><p>ELSTs present with the following symptoms and signs:</p><ul>
- +<li>hearing loss: 95 %, acute in 43% and stepwise in the second half</li>
-</ul><h5>Associations</h5><p>Most often associated with <a href="/articles/von-hippel-lindau-disease-vhl">von Hippel-Lindau disease (vHL)</a>. ELSTs are detected in 11-16% patients with vHL <sup>ref</sup>. In almost 60% of patients with VHL and vestibulocochlear symptoms, there is no evidence on imaging of an endolymphatic-sac tumor <sup>ref</sup>. </p><p>30% of tumours in vHL patients are bilateral.</p><h4>Radiographic features</h4><h5>CT</h5><p>When visible, it gives a picture of bone erosion and the "moth-eaten" petrous bone. ELSTs commonly enhance intensely on CT.</p><h5>MRI</h5><p>Signal characteristics include</p><ul>- +</ul><h5>Associations</h5><p>Most often associated with <a href="/articles/von-hippel-lindau-disease-vhl">von Hippel-Lindau disease (vHL)</a>. ELSTs are detected in 11-16% patients with vHL <sup>ref</sup>. In almost 60% of patients with vHL and vestibulocochlear symptoms, there is no evidence on imaging of an endolymphatic sac tumour <sup>ref</sup>. </p><p>30% of tumours in vHL patients are bilateral.</p><h4>Radiographic features</h4><h5>CT</h5><p>When visible, it gives a picture of bone erosion and the "moth-eaten" petrous bone. ELSTs commonly enhance intensely on CT.</p><h5>MRI</h5><p>Signal characteristics include:</p><ul>
-<strong>T1 C+ (Gd):</strong> typically show enhancement in the non-cystic component of the tumour.</li>- +<strong>T1 C+ (Gd):</strong> typically show enhancement in the non-cystic component of the tumour</li>
-<strong>T2:</strong> often of heterogenous signal</li>- +<strong>T2:</strong> often of heterogeneous signal</li>
Unable to process the form. Check for errors and try again.