Three sporadic cases of endolymphatic sac tumor

Jegannathan, Devimeenal; Kathirvelu, Gopinathan; Mahalingam, Alamelu

doi:10.4103/0028-3886.193816

Cited by 6 publications

(5 citation statements)

References 3 publications

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“…1) [38][39][40][41]. Magnetic resonance imaging (MRI) usually reveals a heterogeneous mass which is hyperintense on contrast enhanced T1 and T2 sequences due to hypervascularity [38,42].…”

Section: Clinical and Radiographic Featuresmentioning

confidence: 99%

“…1) [38][39][40][41]. Magnetic resonance imaging (MRI) usually reveals a heterogeneous mass which is hyperintense on contrast enhanced T1 and T2 sequences due to hypervascularity [38,42]. The heterogeneous appearance may be due to blood products, cysts, cholesterol crystals, calcifications, and flow voids due to vessels [38].…”

Section: Clinical and Radiographic Featuresmentioning

confidence: 99%

“…Magnetic resonance imaging (MRI) usually reveals a heterogeneous mass which is hyperintense on contrast enhanced T1 and T2 sequences due to hypervascularity [38,42]. The heterogeneous appearance may be due to blood products, cysts, cholesterol crystals, calcifications, and flow voids due to vessels [38]. Digital subtraction angiography is helpful for displaying the tumor's vascular structure and supply, which is often complex and may help to map pre-surgical landmarks and to differentiate ELST from other lesions [43].…”

Section: Clinical and Radiographic Featuresmentioning

confidence: 99%

“…The differential diagnosis includes middle ear adenoma, middle ear adenocarcinoma, jugulotympanic paraganglioma, choroid plexus papilloma, non-specific ceruminal gland adenocarcinoma, and metastatic adenocarcinoma (particularly thyroid and renal primaries) which typically show greater nuclear irregularities [14,38,[44][45][46]. Diaz et al reported on 134 temporal bone lesions evaluated at the University of California-Davis between 1994 and 2005: paragangliomas, 78%; jugular foremen schwannomas, 4%; hemangiomas 4%; 7th nerve schwannomas, 4%; endolymphatic sac lesions, 4%; metastases, 3%; middle ear adenoma, 1%; and chondrosarcoma, 1% [43].…”

Section: Pathologic Featuresmentioning

confidence: 99%

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Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone

et al. 2018

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An endolymphatic sac tumor (ELST) is a rare, indolent but locally aggressive tumor arising in the posterior petrous ridge. Patients present with sensorineural hearing loss and tinnitus. As the tumor progresses, patients may experience vertigo, ataxia, facial nerve paresis, pain and otorrhea. Most patients present in their 4th or 5th decade with a wide age range. Patients with von Hippel-Lindau disease have an increased likelihood of developing ELST. Histologically, ELST is a low-grade adenocarcinoma. As it progresses, it destroys bone and extends into adjacent tissues. The likelihood of regional or distant metastases is remote. The optimal treatment is resection with negative margins. Patients with positive margins, gross residual disease, or unresectable tumor are treated with radiotherapy or radiosurgery. Late recurrences are common, so long follow-up is necessary to assess efficacy. The likelihood of cure depends on tumor extent and is probably in the range of 50-75%.

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“…1) [38][39][40][41]. Magnetic resonance imaging (MRI) usually reveals a heterogeneous mass which is hyperintense on contrast enhanced T1 and T2 sequences due to hypervascularity [38,42].…”

Section: Clinical and Radiographic Featuresmentioning

confidence: 99%

Section: Clinical and Radiographic Featuresmentioning

confidence: 99%

Section: Clinical and Radiographic Featuresmentioning

confidence: 99%

Section: Pathologic Featuresmentioning

confidence: 99%

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Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone

et al. 2018

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“…An incidence of ELST of 1:30,000 for the adult population has been reported (5), with 10 to 15% of cases associated with VHL (6). The reported prevalence of ELST in VHL varies, with one study identifying a prevalence as low as 3.6% (7).…”

Section: Introductionmentioning

confidence: 99%

Impact of an Endolymphatic Sac Tumor on Subjective and Objective Audiovestibular Function

2022

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Objective: This study reports subjective and objective audiovestibular function in patients diagnosed with an endolymphatic sac tumor (ELST), to evaluate the clinical implication of vestibular testing in ELST patients. Study Design: Retrospective cohort study. Setting: Tertiary referral center/university hospital. Patients: Five patients diagnosed with unilateral ELST. Intervention: Subjective and objective audiovestibular tests; pure-tone average; speech discrimination score; the Video Head Impulse Test; the cervical vestibular evoked myogenic potential test; the caloric irrigation test; the Speech, Spatial and Qualities of Hearing Scale; the Tinnitus Handicap Inventory; and the Dizziness Handicap Inventory. Main Outcome Measure: Findings and characteristics of both subjective and objective audiovestibular functions.Results: The mean pure-tone average and the mean speech discrimination score were 49 dB HL and 68% on the tumor ear and 13 dB HL and 99% contralaterally. The mean Speech, Spatial and Qualities of Hearing Scale score was 84, and the mean Tinnitus Handicap Inventory score was 64. Caloric testing showed a mean unilateral weakness of 80%. The Video Head Impulse Test of the lateral semicircular canal revealed refixation saccades on all tumor ears and an average gain value of 0.75 on the tumor ear versus 0.94 contralaterally. No cervical vestibular evoked myogenic potentials were elicited on the tumor side, whereas all but one was elicited contralaterally. The average Dizziness Handicap Inventory score was 47. Conclusion: A characteristic pattern of abnormal caloric findings, refixation saccades, and normal vestibulo-ocular reflex gain alongside sensorineural hearing loss on the tumor ear was identified. Asymmetric sensorineural hearing loss in any patient should lead to objective vestibular evaluation to strengthen not only a targeted tumor surveillance strategy and the indication for imaging, but also the design of vestibular rehabilitation. In addition, vestibular findings may guide the choice of surgical approach.

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