A subscription to JoVE is required to view this content. Sign in or start your free trial.
Here, we discuss clinical and radiological features of endolymphatic sac tumors and report the methodology and results of a surgical removal in a case in care at our otologic referral center.
Endolymphatic sac tumors (ELST) are low-grade papillary adenocarcinoma originating from the endolymphatic sac. Usually slow-growing, with local aggressiveness and a low risk of distant metastases, ELST can be sporadic but also frequently associated with von Hippel Lindau disease. The current treatment of ELST is primarily surgical resection. A 55-year-old woman accessed our otologic tertiary level referral center for a sudden worsening of hearing loss in her left ear and vertigo. A magnetic resonance (MRI) and computer tomography scan study subsequently showed a mass in the petrous bone; hence, the presence of an ELST was hypothesized. After embolization of the mass, the patient underwent surgical removal of the lesion. The resection of the mass was done through a translabirinthine approach, with an uneventful procedure. No residual disease remained after surgery. After 24 months of radiologic follow up with MRI, there are no signs of recurrence disease. This paper reports the management of this sporadic ELST, as well as the follow up results, providing clinicians this protocol for the handling of such a challenging otologic skull base surgery and rare disease.
Endolymphatic sac tumors (ELST) are neoplasms originating from the endolymphatic sac, a neuroectoderm-derived organ located in the posteromedial surface of the temporal bone. Histologically, ELST are characterizable as low-grade papillary adenocarcinoma1. Usually, ELST are slow-growing, with local aggressiveness and a low risk of distant metastases2,3,4. ELST can be sporadic, but they are also frequently associated with von Hippel Lindau disease (VHL)4.
Given the slow growing rate, the paucity of the ....
The local ethical committee approved the publication of this clinical case. A written informed consent form was collected from the patient.
1. Clinical and audiological evaluation
From the audiologic assessment, it was possible to identify a moderately severe left sensorineural hearing loss (Figure 1), with a reduction of word discrimination. The tympanometry was bilaterally normal, and the stapedial reflexes were evocable with ipsilateral and contralateral stimulus. At the videonystagmography , it was possible to assess a grade II horizontal spontaneous nystagmus beating on the right side, which grew in intensity after head shaking. From the clinical and audiological.......
ELST are rare and locally aggressive neoplasms that arise on the posteromedial surface of the temporal bone. Due to their slow growth rate, and the initial paucity of the symptoms, the diagnosis is often delayed, resulting in an advanced tumor stage. Since the surgery morbidity as well as the oncologic outcomes are strongly correlated with the tumor dimensions and extension6,8,13,15, an early d.......
None.
....Name | Company | Catalog Number | Comments |
AC40 | Interacoustic | Clinical audiometer | |
AT235 | Interacoustic | Clinical tympanometry | |
Innova 3131 | GE Healthcare | Biplane angiograph | |
LightSpeed VCT | GE Healthcare | 64 Slice CT system | |
S2 πDrive | Striker | Drilling system for Otosurgery | |
Signa HDX | GE Healthcare | 1.5 T MRI system | |
SYNAPSYS VNG | Inventis | Video nystagmograph |
This article has been published
Video Coming Soon
ABOUT JoVE
Copyright © 2024 MyJoVE Corporation. All rights reserved