Endolymphatic sac tumors: experience of three cases

Bastier, Pierre-Louis; Monès, Erwan de; Marro, Magali; Elkhatib, Wael; Franco-Vidal, V.; Liguoro, Dominique; Darrouzet, V.

doi:10.1007/s00405-012-2298-7

Cited by 12 publications

(7 citation statements)

References 22 publications

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“…In general, the smaller the tumor at the time of removal, the lower the postoperative morbidity, where extensive surgery for much larger tumors is associated with disruption of the fallopian canal, jugular foramen, petrous apex, cerebrospinal fluid leak or involvement of the posterior cranial fossa, with concurrent loss of cranial nerve function (specifically VII, IX, and X) [29,33,38,57,58]. Thus, while many clinicians seem to eschew genetic counseling or testing in these patients, identification of the potential genetic relationship and removing the tumors at the earliest, and therefore smallest size possible, is advocated [5,31,33,57,58].…”

Section: Discussionmentioning

confidence: 99%

CAIX and pax-8 Commonly Immunoreactive in Endolymphatic Sac Tumors: A Clinicopathologic Study of 26 Cases with Differential Considerations for Metastatic Renal Cell Carcinoma in von Hippel-Lindau Patients

Thompson

Magliocca

Andreasen

et al. 2018

Head and Neck Pathol

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Endolymphatic sac tumors (ELSTs) are rare, slowly growing temporal bone neoplasms which show a high association with von Hippel-Lindau (VHL) syndrome. The immunohistochemistry evaluation of these papillary-cystic neoplasms frequently raises the differential diagnosis with renal cell carcinoma, among other metastatic neoplasms, whether in VHL patients or not. A cohort of 26 patients with ELSTs were evaluated for histologic features, immunohistochemistry findings, and association with VHL. Standard immunohistochemistry evaluation was performed. Sixteen females and 10 males ranging in age from 10 to 69 years (mean 44; VHL mean: 32) at initial presentation, comprised the cohort of patients. Most (86%) experienced hearing changes or inner ear symptoms (vertigo, dizziness), with an average duration of symptoms for 39 months (range 2-240 months). The tumors were an average of 2.9 cm (range 0.4-8 cm), with 14 left, 11 right sided and one bilateral tumor. Nine patients had documented VHL, with 3 patients having a concurrent or subsequent clear cell renal cell carcinoma. Patients were followed an average of 6.2 years (available in 24 patients): 19 alive without disease, 7.5 years; 2 dead without disease, 1.2 years; and 3 alive with disease, 3.1 years. The neoplastic cells show the following immunohistochemistry findings: AE1/AE3, EMA, CK7, CAIX, GLUT1, VEGF: 100% of cases tested were positive; pax-8: 85% of cases positive; CD10 and RCC: 0% of cases reactive. Based on this cohort of 26 patients with ELST, 9 of whom had VHL, the strong pax-8 and CAIX should be used in conjunction with negative CD10 and RCC to help exclude a metastatic renal cell carcinoma. As CAIX is an enzyme overexpressed in hypoxia and hypoxia inducible factor is what VHL protein regulates, this is an expected, although previously unreported finding. Whether part of VHL or not, VHL mutations may be a somatic rather than germline finding in the tumors, a possible further explanation for the CAIX reaction.

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Section: Discussionmentioning

confidence: 99%

CAIX and pax-8 Commonly Immunoreactive in Endolymphatic Sac Tumors: A Clinicopathologic Study of 26 Cases with Differential Considerations for Metastatic Renal Cell Carcinoma in von Hippel-Lindau Patients

Thompson

Magliocca

Andreasen

et al. 2018

Head and Neck Pathol

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“…Theoretically, we have to rule out intratumoral hemorrhage, which would disappear after 4 months on FLAIR, but contrasted T1 images would still show hyperintensity. Bastier et al 22 reported characteristic MRI imaging of 3 endolymphatic sac tumors, those are presence of cysts and spontaneous hemorrhage, hyperintense on T1-weighted images, hypointense bone spicules and flow void on T1- and T2-Weighted images, and heterogeneous enhancement after injection at MRI. Jeong et al also observed 9 patients of intralabyrinthine schwannoma can display characteristic MRI findings of a lack of normal fluid density on T2-weightd imaging, with corresponding enhancement observed on gadolinium-enhanced T1-weighted scans.…”

Section: Discussionmentioning

confidence: 99%

The Natural History of Labyrinthine Hemorrhage in Patients With Sudden Sensorineural Hearing Loss

et al. 2019

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To investigate the application of inner ear 3-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI) in patients with sudden sensorineural hearing loss (SSNHL) accompanied by inner ear hemorrhage. A total of 1252 SSNHL patients who were admitted from January 2010 to April 2018 were included in the study. The patients' clinical features, complete blood counts, coagulation profiles, audiometry data, and MRI scans were retrospectively reviewed. Twenty-four patients had high labyrinth signals on inner ear 3D-FLAIR MRI (24/1252, 1.9%) that were diagnosed as inner ear hemorrhage. One patient had endolymphatic hydrops on the contralesional side. In the 24 patients, pure tone audiometry curves revealed profound deafness (19/24) and flat moderate hearing loss (5/24); most patients had associated vertigo (23/24) and tinnitus (19/24). Patients with SSNHL (N ¼ 24) were treated. Sixteen patients had invalid improvement, 3 patients were markedly improved, 4 patients had effective treatment, and only 1 patient was cured, for a therapeutic efficacy of 33.3% (8/24). Follow-up 3D-FLAIR MRI in patients showed absorbance of labyrinthine hemorrhage and disappearance of the high signal intensity in the inner ear within 2 weeks to 4 months. Inner ear 3D-FLAIR MRI indicate that most cases of inner ear hemorrhage are spontaneous and that high labyrinth signals are absorbed within 4 months. The site of labyrinth hemorrhage is irregular and independent of hearing loss. Conventional treatment is not very effective, and an appropriate therapy for SSNHL requires further investigation.

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“…Its morphological features often seem to be benign, but locally invasive characteristic and destruction of the temporal bone disclose the low grade malignant nature of the tumor. ELSTs have a tendency to recur, but only two cases of metastasis have been reported and there has been no death. In this group of 10 cases, all of them had temporal bone destruction; five cases had recurrence and one of these cases recurred three times.…”

Section: Discussionmentioning

confidence: 99%

Clinicopathologic study of endolymphatic sac tumor (ELST) and differential diagnosis of papillary tumors located at the cerebellopontine angle

Wang

Cui

et al. 2015

Neuropathology

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We investigated the clinicopathologic features and immunophenotypes of 10 cases of endolymphatic sac tumor (ELST) and compared them with other papillary tumors, including eight cases of choroid plexus papilloma (CPP), three cases of atypical choroid plexus papilloma (ACPP), two cases of papillary ependymoma (PE), three cases of papillary meningioma (PM) and two cases of metastatic carcinoma (MC) the at cerebellopontine angle (CPA). The age at onset of ELST ranged from 13 to 39 years. The male-to-female ratio was 1:1. The clinical presentations were primarily ear-related symptoms. The temporal bones showed extensive destruction. Histologically, the important characteristics for differential diagnosis with CPP, which is most similar to ELST, include the quantity of blood vessels, the nuclei location at apical surface of the papillary, clear cytoplasm cells sometimes with visible vacuoles, psammoma bodies and dura or bone invasion. Immunohistochemistry stains for AE1/AE3, cytokeratin CK)5/6, epithelial membrane antigen, CK8/18, S-100, and synaptophysin are helpful in diagnosis of ELST. In ELST, ultrastructure of uniform 2 μm vesicles in cytoplasm was seen, and gene analysis also showed missense mutation in exon 3. This study indicates that the above histological features combined with immunohistochemistry findings are important for making the correct diagnosis. Gene analysis should be used in patients without medical history to exclude von Hippel-Lindau disease.

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Endolymphatic sac tumors: experience of three cases

Cited by 12 publications

References 22 publications

CAIX and pax-8 Commonly Immunoreactive in Endolymphatic Sac Tumors: A Clinicopathologic Study of 26 Cases with Differential Considerations for Metastatic Renal Cell Carcinoma in von Hippel-Lindau Patients

CAIX and pax-8 Commonly Immunoreactive in Endolymphatic Sac Tumors: A Clinicopathologic Study of 26 Cases with Differential Considerations for Metastatic Renal Cell Carcinoma in von Hippel-Lindau Patients

The Natural History of Labyrinthine Hemorrhage in Patients With Sudden Sensorineural Hearing Loss

Clinicopathologic study of endolymphatic sac tumor (ELST) and differential diagnosis of papillary tumors located at the cerebellopontine angle

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