CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2021; 40(04): e387-e393
DOI: 10.1055/s-0041-1730419
Case Report | Relato de Caso

Endolymphatic Sac Tumour: A Case Report and Review of the Literature

Tumor do saco endolinfático: relato de caso e revisão da literatura
1   Department of Neurosurgery, Neuron Hospital, Vadodara, Gujarat, India
,
Ranjan Aiyer
2   Department of Ear, Nose and Throat, Baroda Medical College and SSG Hospital, Vadodara, Gujarat, India
,
Srinivasan Chamarajan
3   Department of Pathology, Sterling Hospital, Vadodara, Gujarat, India
,
Nidhi Vala
3   Department of Pathology, Sterling Hospital, Vadodara, Gujarat, India
› Author Affiliations
Funding The authors confirm that they have not received any funding for this study from any source.

Abstract

Introduction Endolymphatic sac tumor (ELST) is a slow-growing, low-grade, locally-infiltrative tumor arising from the endolymphatic sac/duct, which is located in the posterior part of the petrous temporal bone. It may be sporadic in origin, or may be associated with Von-Hippel Lindau (VHL) syndrome.

Case description A 40-year-old female patient with an ELST without VHL syndrome who was treated successfully by microsurgical extirpation of the tumor.

Discussion We discuss the radiological features and the histopathology of this rare tumor and review the relevant literature.

Conclusion The case herein reported adds to the previously-reported cases of this rare tumor.

Resumo

Introdução O tumor do saco endolinfático (ELST) é um tumor localmente infiltrativo, de baixo grau e crescimento lento, originado do ducto/saco endolinfático, que está localizado na parte posterior do osso petroso temporal. Pode ser de origem esporádica ou pode estar associado à síndrome de Von-Hippel Lindau (VHL).

Descrição do caso Paciente do sexo feminino, 40 anos, com ELST sem síndrome de VHL, tratada com sucesso por extirpação microcirúrgica do tumor.

Discussão Discutimos as características radiológicas e a histopatologia desse tumor raro e revisamos a literatura relevante.

Conclusão O caso aqui relatado se soma aos casos previamente relatados desse tumor raro.

Declarations

We the authors confirm that neither this manuscript nor parts of it have been submitted for publication anywhere.


Ethics approval and consent to participate

The case report was presented before the Neuron Hospital Ethics Committee, which, after due review and deliberation, has given the consent for the publication of this article on June 1st, 2020.


Consent for publication

We the authors give consent to Brazilian Neurosurgery to publish our study. We also confirm that consent for publication of the case and photographs has been obtained from the patient.


Availability of data and materials

The present study does not contain any data or materials from other sources. The data supporting the results can be found in the hospitals where the surgery was performed, namely the SSG Hospital and Sterling hospital, Vadodara Gujarat, India.


Authors' Contributions

Dr. APP was involved involved in treating the patient, and in preparing the manuscript and its submission. Dr. RA was involved in treating the patient, and contributed to the operative technique section. Dr. SC and Dr. NV were the pathologists who provided the pathological diagnosis, and they contributed to the sections on histopathology and immunohistochemistry, and took the photographs.




Publication History

Received: 31 October 2020

Accepted: 09 March 2021

Article published online:
13 August 2021

© 2021. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Hassard AD, Boudreau SF, Cron CC. Adenoma of the endolymphatic sac. J Otolaryngol 1984; 13 (04) 213-216
  • 2 Heffner DK. Low-grade adenocarcinoma of probable endolymphatic sac origin: a clinicopathologic study of 20 cases. Cancer 1989; 64: 2292-3022
  • 3 Megerian CA, McKenna MJ, Nuss RC. et al. Endolymphatic sac tumors: histopathologic confirmation, clinical characterization, and implication in von Hippel-Lindau disease. Laryngoscope 1995; 105 (8 Pt 1): 801-808
  • 4 Wick CC, Manzoor NF, Semaan MT, Megerian CA. Endolymphatic sac tumors. Otolaryngol Clin North Am 2015; 48 (02) 317-330
  • 5 Zanoletti E, Girasoli L, Borsetto D, Opocher G, Mazzoni A, Martini A. Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies. Acta Otorhinolaryngol Ital 2017; 37 (05) 423-429 DOI: 10.14639/0392-100X-1402.
  • 6 Tay KY, Yu E, Kassel E. Spinal metastasis from endolymphatic sac tumor. AJNR Am J Neuroradiol 2007; 28 (04) 613-614
  • 7 Bambakidis NC, Rodrigue T, Megerian CA, Ratcheson RA. Endolymphatic sac tumor metastatic to the spine. Case report. J Neurosurg Spine 2005; 3 (01) 68-70
  • 8 Lo WW, Applegate LJ, Carberry JN. et al. Endolymphatic sac tumors: radiologic appearance. Radiology 1993; 189 (01) 199-204
  • 9 Mukherji SK, Albernaz VS, Lo WW. et al. Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients. Radiology 1997; 202 (03) 801-808
  • 10 Bambakidis NC, Megerian CA, Ratcheson RA. Differential grading of endolymphatic sac tumor extension by virtue of von Hippel-Lindau disease status. Otol Neurotol 2004; 25 (05) 773-781
  • 11 Friedman RA, Hoa M, Brackmann DE. Surgical management of endolymphatic sac tumors. J Neurol Surg B Skull Base 2013; 74 (01) 12-19 DOI: 10.1055/s-0032-1329622.
  • 12 Kefeli AU, Yilmaz M, Sengoz M, Peker S. Gamma Knife Radiosurgery for Tumors of the Endolymphatic Sac. Turk Neurosurg 2017; 27 (04) 665-669