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Skull Base 2007; 17(6): 379-393
DOI: 10.1055/s-2007-991116
ORIGINAL ARTICLE

© Thieme Medical Publishers

Tumors and Pseudotumors of the Endolymphatic Sac

Rodney C. Diaz1 , Esmael H. Amjad2 , Eric W. Sargent3 , Michael J. LaRouere3 , Wayne T. Shaia4
  • 1Department of Otolaryngology-Head and Neck Surgery, University of California Davis Medical Center, Sacramento, California
  • 2Department of Otolaryngology-Head and Neck Surgery, Wayne State University, Detroit Medical Center, Detroit, Michigan
  • 3Michigan Ear Institute, Farmington Hills, Michigan
  • 4Balance and Ear Center, Richmond, Virginia
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Publication History

Publication Date:
18 October 2007 (online)

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ABSTRACT

This article reports on the presentation, diagnosis, management, and treatment outcomes of lesions of the endolymphatic sac in patients treated at a tertiary neurotology referral center. It summarizes survival results in the largest series groups and presents a new diagnostic entity of pseudotumor of the endolymphatic sac. The study includes retrospective review of all patients diagnosed with lesions of the endolymphatic sac within our practice between 1994 and 2005 as well as review of the literature. The primary outcome measure was survival, and the secondary outcome measure was disease-free survival following definitive resection. Postoperative complications were assessed. Survival characteristics of the largest reported case series groups were reviewed. Five cases of endolymphatic sac lesions were identified. Of these, three were true endolymphatic sac tumors and two were inflammatory pseudotumors of the endolymphatic sac. All three of the endolymphatic sac tumors patients survived (100%), and two of the three had disease-free survival (67%). Two of three patients maintained persistent facial paresis postoperatively. Both patients with benign pseudotumors survived (100%). Our study concluded that endolymphatic sac tumors are rare neoplasms of the temporal bone that, although locally aggressive and invasive, have excellent prognosis for survival with complete resection. We report a new entity of pseudotumor of the endolymphatic sac that mimics true sac tumors in every respect on presentation but which is non-neoplastic in origin.

KEYWORDS

Endolymphatic sac tumor - hydrops - papillary adenoma - pseudotumor - von Hippel-Lindau disease

REFERENCES

  • 1 Hassard A D, Boudreau S F, Cron C C. Adenoma of the endolymphatic sac.  J Otolaryngol. 1984;  13 213-216
    PubMedGoogle Scholar
  • 2 Heffner D K. Low-grade adenocarcinoma of probable endolymphatic sac origin: a clinicopathologic study of 20 cases.  Cancer. 1989;  64 2292-2302
    CrossrefPubMedGoogle Scholar
  • 3 Stanley J, Pickett B. Endolymphatic sac tumors. In: Jackler RK Tumors of the Ear and Temporal Bone. Philadelphia; Lippincott Williams and Wilkins 2000: 156
    Google Scholar
  • 4 Lo W W, Applegate L J, Carberry J N et al.. Endolymphatic sac tumors: radiologic appearance.  Radiology. 1993;  189 199-204
    PubMedGoogle Scholar
  • 5 Megerian C A, McKenna M J, Nuss R C et al.. Endolymphatic sac tumors: histopathologic confirmation, clinical characterization, and implication in von Hippel-Lindau disease.  Laryngoscope. 1995;  105 801-808
    CrossrefPubMedGoogle Scholar
  • 6 Benecke Jr J E, Noel F L, Carberry J N, House J W, Patterson M. Adenomatous tumors of the middle ear and mastoid.  Am J Otol. 1990;  11 20-26
    PubMedGoogle Scholar
  • 7 Feghali J G, Levin R J, Llena J, Bradley M K, Kantrowitz A B. Aggressive papillary tumors of the endolymphatic sac: clinical and tissue culture characteristics.  Am J Otol. 1995;  16 778-782
    PubMedGoogle Scholar
  • 8 Hansen M R, Luxford W M. Surgical outcomes in patients with endolymphatic sac tumors.  Laryngoscope. 2004;  114 1470-1474
    CrossrefPubMedGoogle Scholar
  • 9 Rodrigues S, Fagan P, Turner J. Endolymphatic sac tumors: a review of the St. Vincent's hospital experience.  Otol Neurotol. 2004;  25 599-603
    CrossrefPubMedGoogle Scholar
  • 10 Kim H J, Butman J A, Brewer C et al.. Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease: implications for their natural history, diagnosis, and treatment.  J Neurosurg. 2005;  102 503-512
    CrossrefPubMedGoogle Scholar
  • 11 Megerian C A, Haynes D S, Poe D S, Choo D I, Keriakas T J, Glasscock III M E. Hearing preservation surgery for small endolymphatic sac tumors in patients with von Hippel-Lindau syndrome.  Otol Neurotol. 2002;  23 378-387
    CrossrefPubMedGoogle Scholar
  • 12 Kempermann G, Neumann H P, Volk B. Endolymphatic sac tumours.  Histopathology. 1998;  33 2-10
    CrossrefPubMedGoogle Scholar
  • 13 Roche P H, Dufour H, Figarella-Branger D, Pellet W. Endolymphatic sac tumors: report of three cases.  Neurosurgery. 1998;  42 927-932
    PubMedGoogle Scholar
  • 14 Forrest A W, Turner J J, Fagan P A. Aggressive papillary middle ear tumour.  J Laryngol Otol. 1991;  105 950-953
    PubMedGoogle Scholar
  • 15 Megerian C A, Pilch B Z, Bhan A K, McKenna M J. Differential expression of transthyretin in papillary tumors of the endolymphatic sac and choroid plexus.  Laryngoscope. 1997;  107 216-221
    PubMedGoogle Scholar
  • 16 Lonser R R, Kim H J, Butman J A, Vortmeyer A O, Choo D I, Oldfield E H. Tumors of the endolymphatic sac in von Hippel-Lindau disease.  N Engl J Med. 2004;  350 2481-2486
    CrossrefPubMedGoogle Scholar
  • 17 Noujaim S E, Pattekar M A, Cacciarelli A, Sanders W P, Wang A M. Paraganglioma of the temporal bone: role of magnetic resonance imaging versus computed tomography.  Top Magn Reson Imaging. 2000;  11 108-122
    CrossrefPubMedGoogle Scholar
  • 18 Patel N P, Wiggins III R H, Shelton C. The radiologic diagnosis of endolymphatic sac tumors.  Laryngoscope. 2006;  116 40-46
    CrossrefPubMedGoogle Scholar
  • 19 Shin J H, Lee H K, Jeong A K, Park S H, Choi C G, Suh D C. Choroid plexus papilloma in the posterior cranial fossa: MR, CT, and angiographic findings.  Clin Imaging. 2001;  25 154-162
    CrossrefPubMedGoogle Scholar
  • 20 Mukherji S K, Albernaz V S, Lo W W et al.. Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients.  Radiology. 1997;  202 801-808
    CrossrefPubMedGoogle Scholar
  • 21 Wagle V, Melanson D, Ethier R, Bertrand G, Villemure J G. Choroid plexus papilloma: magnetic resonance, computed tomography, and angiographic observations.  Surg Neurol. 1987;  27 466-468
    CrossrefPubMedGoogle Scholar
  • 22 Gadre A K, Fayad J N, O'Leary M J, Zakhary R, Linthicum Jr F H. Arterial supply of the human endolymphatic duct and sac.  Otolaryngol Head Neck Surg. 1993;  108 141-148
    PubMedGoogle Scholar
  • 23 Neumann H P, Wiestler O D. Clustering of features of von Hippel-Lindau syndrome: evidence for a complex genetic locus.  Lancet. 1991;  337 1052-1054
    CrossrefPubMedGoogle Scholar
  • 24 Bambakidis N C, Megerian C A, Ratcheson R A. Differential grading of endolymphatic sac tumor extension by virtue of von Hippel-Lindau disease status.  Otol Neurotol. 2004;  25 773-781
    CrossrefPubMedGoogle Scholar
  • 25 Latif F, Tory K, Gnarra J et al.. Identification of the von Hippel-Lindau disease tumor suppressor gene.  Science. 1993;  260 1317-1320
    CrossrefPubMedGoogle Scholar
  • 26 Kim W Y, Kaelin W G. Role of VHL gene mutation in human cancer.  J Clin Oncol. 2004;  22 4991-5004
    CrossrefPubMedGoogle Scholar
  • 27 Vortmeyer A O, Choo D, Pack S D, Oldfield E, von Zhuang Z. Hippel-Lindau disease gene alterations associated with endolymphatic sac tumor.  J Natl Cancer Inst. 1997;  89 970-972
    PubMedGoogle Scholar
  • 28 Manski T J, Heffner D K, Glenn G M et al.. Endolymphatic sac tumors: a source of morbid hearing loss in von Hippel-Lindau disease.  JAMA. 1997;  277 1461-1466
    CrossrefPubMedGoogle Scholar
  • 29 Vortmeyer A O, Huang S C, Koch C A et al.. Somatic von Hippel-Lindau gene mutations detected in sporadic endolymphatic sac tumors.  Cancer Res. 2000;  60 5963-5965
    PubMedGoogle Scholar
  • 30 Chen F, Kishida T, Yao M et al.. Germline mutations in the von Hippel-Lindau disease tumor suppressor gene: correlations with phenotype.  Hum Mutat. 1995;  5 66-75
    CrossrefPubMedGoogle Scholar
  • 31 Ferreira M A, Feiz-Erfan I, Zabramski J M, Spetzler R F, Coons S W, Preul M C. Endolymphatic sac tumor: unique features of two cases and review of the literature.  Acta Neurochir (Wien). 2002;  144 1047-1053
    PubMedGoogle Scholar
  • 32 Bambakidis N C, Rodrigue T, Megerian C A, Ratcheson R A. Endolymphatic sac tumor metastatic to the spine: case report.  J Neurosurg Spine. 2005;  3 68-70
    PubMedGoogle Scholar
  • 33 Hashimoto M, Yokota A, Urasaki E, Imada H, Yamamoto H. Surgical treatment of endolymphatic sac tumor with adjunctive stereotactic radiation therapy: case report.  Neurol Med Chir (Tokyo). 2004;  44 595-599
    CrossrefPubMedGoogle Scholar
  • 34 Zechner G, Altmann F. Histological studies on the human endolymphatic duct and sac.  Pract Otorhinolaryngol (Basel). 1969;  31 65-83
    PubMedGoogle Scholar
  • 35 Ikeda M, Sando I. Endolymphatic duct and sac in patients with Meniere's disease: a temporal bone histopathological study.  Ann Otol Rhinol Laryngol. 1984;  93 540-546
    PubMedGoogle Scholar
  • 36 Schindler R A. The ultrastructure of the endolymphatic sac in man.  Laryngoscope. 1980;  90 1-39
    PubMedGoogle Scholar
  • 37 Schindler R A. Histopathology of the human endolymphatic sac.  Am J Otol. 1981;  3 139-143
    PubMedGoogle Scholar
  • 38 Fitzgerald D C, Mark A S. Endolymphatic duct/sac enhancement on gadolinium magnetic resonance imaging of the inner ear: preliminary observations and case reports.  Am J Otol. 1996;  17 603-606
    PubMedGoogle Scholar

Rodney C DiazM.D. 

Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, University of California Davis Medical Center

2521 Stockton Boulevard, Suite 7200, Sacramento, CA 95817

Email: rcdiaz@ucdavis.edu

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