Skip to main content

Advertisement

Log in

Grading system and surgical approaches for endolymphatic sac tumors

  • Otology
  • Published:
European Archives of Oto-Rhino-Laryngology Aims and scope Submit manuscript

Abstract

Purpose

Endolymphatic sac tumors (ELSTs) are rare, low-grade adenocarcinomas arising from the endolymphatic sac. This study aims to present a novel grading system for ELSTs to determine the optimal management strategy.

Methods

We performed a retrospective analysis of 16 patients with 17 ELSTs. The tumor location and involved adjacent neurovascular structures on CT and MRI were selected to establish the grading system.

Results

Based on the novel grading system, grade III a tumors were most common (7/17), followed by grade I (4/17), grade II (3/17), and grade III b (3/17) tumors. Eight advanced ELSTs (grade III a and III b) received an infra-temporal fossa approach, while the other 6 early stage ELSTs (grade I and II) underwent either a retrolabyrinthine approach with posterior petrosectomy or a translabyrinthine approach combined with subtotal temporal bone resection. Hearing preservation was achieved in 2 grade I patients. Postoperative facial nerve function was HB II in 1 grade III a patient who underwent anterior facial nerve transposition and was HB III in 4 advanced patients who received facial nerve grafts with the great auricular nerve or facial-hypoglossal nerve anastomosis. The mean follow-up time was 35.1 months. Two grade III patients and 1 grade II patient had tumor recurrence during follow-up, among whom 1 grade III b patient had two cases of recurrence.

Conclusion

A correct initial diagnosis was established in all patients after meticulous imaging studies. Surgical resection is still the first choice to manage patients with ELSTs. The novel grading system enables surgeons to select tailored surgical approaches. Long-term follow-up is necessary following surgical intervention.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

Data availability

The raw data used to support this study may be available from the corresponding author upon reasonable request.

References

  1. Megerian CA, McKenna MJ, Nuss RC et al (1995) Endolymphatic sac tumors: histopathologic confirmation, clinical characterization, and implication in von Hippel-Lindau disease. Laryngoscope 105:801–808

    Article  CAS  Google Scholar 

  2. Megerian CA, Semaan MT (2007) Evaluation and management of endolymphatic sac and duct tumors. Otolaryngol Clin N Am 40:463–478

    Article  Google Scholar 

  3. Heffner DK (1989) Low-grade adenocarcinoma of probable endolymphatic sac origin A clinicopathologic study of 20 cases. Cancer 64:2292–2302

    Article  CAS  Google Scholar 

  4. Lonser RR, Kim HJ, Butman JA, Vortmeyer AO, Choo DI, Oldfield EH (2004) Tumors of the endolymphatic sac in von Hippel-Lindau disease. N Engl J Med 350:2481–2486

    Article  CAS  Google Scholar 

  5. Kirsh ER, Kozin ED, Knoll RM et al (2018) Sequential imaging in patient with suspected Meniere's disease identifies endolymphatic sac tumor. Otol Neurotol 39:e856–e859

    Article  Google Scholar 

  6. Patel NP, Wiggins RH 3rd, Shelton C (2006) The radiologic diagnosis of endolymphatic sac tumors. Laryngoscope 116(1):40–46

    Article  Google Scholar 

  7. Butman JA, Nduom E, Kim HJ, Lonser RR (2013) Imaging detection of endolymphatic sac tumor-associated hydrops. J Neurosurg 119:406–411

    Article  Google Scholar 

  8. Kitahara T, Maekawa C, Kizawa K, Kamakura T, Horii A, Inohara H (2011) Endolymphatic sac tumor with overexpression of V2 receptor mRNA and inner ear hydrops. Acta Otolaryngol 131:951–957

    Article  CAS  Google Scholar 

  9. 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

    Article  Google Scholar 

  10. Devaney KO, Ferlito A, Rinaldo A (2003) Endolymphatic sac tumor (low-grade papillary adenocarcinoma) of the temporal bone. Acta Otolaryngol 123:1022–1026

    Article  Google Scholar 

  11. Kupferman ME, Bigelow DC, Carpentieri DF, Bilaniuk LT, Kazahaya K (2004) Endolymphatic sac tumor in a 4-year-old boy. Otol Neurotol 25:782–786

    Article  Google Scholar 

  12. Hansen MR, Luxford WM (2004) Surgical outcomes in patients with endolymphatic sac tumors. Laryngoscope 114:1470–1474

    Article  Google Scholar 

  13. Hou ZH, Huang DL, Han DY, Dai P, Young WY, Yang SM (2012) Surgical treatment of endolymphatic sac tumor. Acta Otolaryngol 132:329–336

    Article  Google Scholar 

  14. Wick CC, Manzoor NF, Semaan MT, Megerian CA (2015) Endolymphatic sac tumors. Otolaryngol Clin N Am 48:317–330

    Article  Google Scholar 

  15. Gaeta M, Blandino A, Minutoli F, Pandolfo I (1999) Sudden unilateral deafness with endolymphatic sac adenocarcinoma: MRI. Neuroradiology 41:799–801

    Article  CAS  Google Scholar 

  16. Jagannathan J, Butman JA, Lonser RR et al (2007) Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report. J Neurosurg 107:421–425

    Article  Google Scholar 

  17. 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

    Article  CAS  Google Scholar 

  18. Li J, Wang M, Sun L et al (2019) The correlation analysis of intralabyrinthine haemorrhage magnetic resonance imaging with hearing loss and prognosis: a retrospective analysis of 207 cases. Clin Otolaryngol 44(6):1096–1100

    Article  Google Scholar 

  19. Mukherji SK, Albernaz VS, Lo WW et al (1997) Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients. Radiology 202:801–808

    Article  CAS  Google Scholar 

  20. Poletti AM, Dubey SP, Barbo R et al (2013) Sporadic endolymphatic sac tumor: its clinical, radiological, and histological features, management, and follow-up. Head Neck 35:1043–1047

    Article  Google Scholar 

  21. Nevoux J, Nowak C, Vellin JF et al (2014) Management of endolymphatic sac tumors: sporadic cases and von Hippel-Lindau disease. Otol Neurotol 35:899–904

    Article  Google Scholar 

Download references

Funding

Not applicable.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Chunfu Dai.

Ethics declarations

Conflict of interest

The authors declare that there is no funding, financial relationships, or conflicts of interest to disclose regarding the publication of this paper.

Ethics approval

This study was approved by the ethics committee of the Eye, Ear, Nose, and Throat Hospital of Fudan University, Shanghai, China (2014007).

Consent to participate

All patients signed informed consent forms.

Consent for publication

All patients and authors approved the publication of this study.

Code availability

Not applicable.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Li, F., Zhang, Y., Li, W. et al. Grading system and surgical approaches for endolymphatic sac tumors. Eur Arch Otorhinolaryngol 278, 1345–1353 (2021). https://doi.org/10.1007/s00405-020-06185-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00405-020-06185-3

Keywords

Navigation