Abstract
Purpose
To review the resections of endolymphatic sac tumor (ELST) and describe our experience in the surgical management of ELST.
Methods
Retrospective investigation of consecutive patients who underwent resection of ELSTs at our hospital between 1999 and 2019. The symptoms, diagnosis, surgical findings, and outcomes were analyzed to develop a tumor staging system and corresponding surgical strategy.
Results
Retrospective review revealed the surgical treatment of 22 ELSTs. Based on intraoperative findings of tumor extent and size, ELSTs were classified into two types. Type-I (n = 6) referred to the small tumors that were locally confined with limited invasion of semicircular canals and dura; type-II (n = 16) referred to the large tumors that presented extensive erosion of at least one anatomic structure apart from the semicircular canals and the dura around endolymphatic sac. In this case series, Type-I ELST is amenable to resection through a transmastoidal approach, and subtotal petrosectomy is appropriate for the resection of type-II ELST. Sensorineural hearing loss (SNHL) is the most commonly preoperative symptom in both two types of cases. Five type-II ELSTs experienced recurrence and underwent reoperation, whereas all type-I ELSTs did not.
Conclusion
ELST usually results in SNHL (95%) at the time of diagnosis. The surgical strategy and prognosis of ELST resections are different between type-I and type-II: type-I ELST is amenable to transmastoidal approach with the preservation of facial nerve, whereas type-II ELST increase the surgical difficulty and the risk of recurrence, and subtotal petrosectomy is the basic requirement for the resection of type-II ELST.
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs00405-022-07447-y/MediaObjects/405_2022_7447_Fig1_HTML.jpg)
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs00405-022-07447-y/MediaObjects/405_2022_7447_Fig2_HTML.jpg)
Similar content being viewed by others
Abbreviations
- ELST:
-
Endolymphatic sac tumor
- SNHL:
-
Sensorineural hearing loss
- yr:
-
Year
- NRT:
-
Neural response telemetry
- CSF:
-
Cerebrospinal fluid
- H–B:
-
House–Brackmann
References
Mendenhall WM, Suárez C, Skálová A et al (2018) Current treatment of endolymphatic sac tumor of the temporal bone. Adv Ther 35(7):887–898
Nevoux J, Nowak C, Vellin JF et al (2014) Management of endolymphatic sac tumors: sporadic cases and von Hippel-Lindau disease. Otol Neurotol 35(5):899–904
Balasubramaniam S, Deshpande RB, Misra BK (2009) Gamma knife radiosurgery in jugular foramen endolymphatic sac adenocarcinoma. J Clin Neurosci 16(5):710–711
Poletti AM, Dubey SP, Colombo G et al (2016) Treatment of endolymphatic sac tumour (Papillary adenocarcinoma) of the temporal bone. Rep Pract Oncol Radiother 21(4):391–394
Bambakidis NC, Megerian CA, Ratcheson RA (2004) Differential grading of endolymphatic sac tumor extension by virtue of von Hippel-Lindau disease status. Otol Neurotol 25(5):773–781
Schipper J, Maier W, Rosahl SK et al (2006) Endolymphatic sac tumours: surgical management. J Otolaryngol 35(6):387–394
Roth TN, Hanebuth D, Probst R (2011) Prevalence of age-related hearing loss in Europe: a review. Eur Arch Otorhinolaryngol 268(8):1101–1107
Chittiboina P, Lonser RR (2015) Von Hippel-Lindau disease Handb Clin Neurol 132:139–156
Gaffey MJ, Mills SE, Fechner RE, et al (1988) Aggressive papillary middle-ear tumor. A clinicopathologic entity distinct from middle-ear adenoma. Am J Surg Pathol 12(10):790–7.
Butman JA, Kim HJ, Baggenstos M et al (2007) Mechanisms of morbid hearing loss associated with tumors of the endolymphatic sac in von Hippel-Lindau disease. JAMA 298(1):41–48
Kim HJ, Hagan M, Butman JA et al (2013) Surgical resection of endolymphatic sac tumors in von Hippel-Lindau disease: findings, results, and indications. Laryngoscope 123(2):477–483
Carlson ML, Thom JJ, Driscoll CL et al (2013) Management of primary and recurrent endolymphatic sac tumors. Otol Neurotol 34(5):939–943
Friedman RA, Hoa M, Brackmann DE (2013) Surgical management of endolymphatic sac tumors. J Neurol Surg B Skull Base 74(1):12–19
Husseini ST, Piccirillo E, Taibah A et al (2013) The Gruppo Otologico experience of endolymphatic sac tumor. Auris Nasus Larynx 40(1):25–31
Zhuang Z, Frerich JM, Huntoon K et al (2014) Tumor derived vasculogenesis in von Hippel-Lindau disease-associated tumors. Sci Rep 4:4102
Lonser RR, Kim HJ, Butman JA et al (2004) Tumors of the endolymphatic sac in von Hippel-Lindau disease. N Engl J Med 350(24):2481–2486
Rodrigues S, Fagan P, Turner J (2004) Endolymphatic sac tumors: a review of the St. Vincent’s hospital experience Otol Neurotol 25(4):599–603
Funding
This study was supported by grant from Innovative Research Group Project of the National Natural Science Foundation of China (81820108009).
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Wu, N., Ma, X., Shen, W. et al. Surgical management of endolymphatic sac tumor: classification, outcomes and strategy. A single institution’s experience. Eur Arch Otorhinolaryngol 280, 69–76 (2023). https://doi.org/10.1007/s00405-022-07447-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00405-022-07447-y