Brain Tumor Pathology

, Volume 16, Issue 2, pp 69–76 | Cite as

A case of endolymphatic sac tumor with long-term survival

  • Kenichiroh Asano
  • Tetsuji Sekiya
  • Toru Hatayama
  • Masahiko Tanaka
  • Atsuhito Takemura
  • Shigeharu Suzuki
  • Osami Kubo
  • Yoshihiro Ishihara
Case Report


A 72-year-old man developed left facial palsy at age 14 and left-sided hearing loss at age 20. At the age of 59, he presented with gait disturbance, and a large left cerebellopontine angle tumor was detected, which had markedly destroyed the pyramidal bone. The tumor was subtotally resected, but he required two more operations at the ages of 64 and 69 because of tumor regrowth. At the present time, recurrent tumor has destroyed the occipital bone and is invading the scalp. However, eventhough he has several cranial nerve palsies and cerebellar ataxia, he remains in stable condition and demonstrates long-term survival. The patient's surgical specimens revealed a papillary adenoma, which was recently thought to be of endolymphatic sac origin, although the origin of this kind of tumor, whether arising from the middle ear or from the endolymphatic sac, has not been established with certainty so far. In this paper, we provide further evidence that this tumor originates from the endolymphatic sac, based on anatomical, histopathological, and embryological evidence.

Key words

Endolymphatic sac tumor Adenoma Papillary structure Immunohistochemistry Long-term survival 


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  1. 1.
    Heffner DK (1989) Low grade adenocarcinoma of probable endolymphatic sac origin: a clinicopathologic study of 20 cases. Cancer 64:2292–2302PubMedCrossRefGoogle Scholar
  2. 2.
    Li JC, Brackmann DE, Lo WWM, et al (1993) Reclassification of aggressive adenomatous mastoid neoplasms as endolymphatic sac tumors. Laryngoscope 103:1342–1348PubMedGoogle Scholar
  3. 3.
    Miura K, Tsutsui Y (1996) A case report of low-grade papillary adenocarcinoma of probable endolymphatic sac origin with a review of the literature and differential diagnosis (in Japanese). Byouri to Rinshyou (Pathol Clin Med) 14:251–258Google Scholar
  4. 4.
    Adam W, Johnson JC, Paul DJ, et al (1982) Primary adenocarinoma of the middle ear. AJNR 3:674–676PubMedGoogle Scholar
  5. 5.
    Glassock ME, McKennan KX, Levine SC, et al (1987) Primary adenocarcinoma of the middle ear and temporal bone. Arch Otolaryngol Head Neck Surg 113:822–824Google Scholar
  6. 6.
    Goebel JA, Smith PG, Kemink JL, et al (1987) Primary adenocarinomas of the temporal bone mimicking paragangliomas: radiographic and clinical recognition. Otolaryngol Head Neck Surg 96:231–238PubMedGoogle Scholar
  7. 7.
    Gulya AJ, Glasscock ME, Pensak ML (1986) Primary adenocarcinoma of the temporal bone with posterior fossa extension: case report. Laryngoscope 96:675–677PubMedGoogle Scholar
  8. 8.
    Schuller DE, Conley JJ, Goodman JH, et al (1983) Primary adenocarcinoma of the middle ear. Otolaryngol Head Neck Surg 91:280–283PubMedGoogle Scholar
  9. 9.
    Stone HE, Lipa M, Bell RD (1975) Primary adenocarcinoma of the middle ear. Arch Otolaryngol 101:702–705PubMedGoogle Scholar
  10. 10.
    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 12:790–797PubMedGoogle Scholar
  11. 11.
    Benecke JE, Noel FL, Carberry LN, et al (1990) Adenomatous tumors of the middle ear and mastoid. Am J Otol 11:20–26PubMedGoogle Scholar
  12. 12.
    Hassard AD, Boudreau SF, Cron CC (1984) Adenoma of the endolymphatic sac. J Otolaryngol 13:213–216PubMedGoogle Scholar
  13. 13.
    Amano H, Kinoshita Y, Ito H, et al (1995) Aggressive papillary adenoma of probable endolymphatic sac origin: report of a case (in Japanese) Jibi Inkoka, Tokeibu Geka (Otolaryngol Head Neck Surg) 67:128–132Google Scholar
  14. 14.
    Folker RJ, Meyerhoff WL, Rushing EJ (1997) Aggressive papillary adenoma of the cerebellopontine angle: case report of an endolymphatic sac tumor. Am J Otolaryngol 18:135–139PubMedCrossRefGoogle Scholar
  15. 15.
    Pollak A, Böhmer A, Spycher M, et al (1996) Are papillary adenomas endolymphatic sac tumors? Ann Otol Laryngol 104:613–619Google Scholar
  16. 16.
    Reijneveld J, Hanlo P, Groenewoud G, et al (1997) Endolymphatic sac tumor: a case report and review of the literature. Surg Neurol 48:368–373PubMedCrossRefGoogle Scholar
  17. 17.
    Lo WWM, Applegate LJ, Carberry JN et al (1993) Endolymphatic sac tumors: radiologic appearance. Radiology 189:199–204PubMedGoogle Scholar
  18. 18.
    Tos M (1984) Anatomy and histology of the middle ear. Clin Rev Allergy 2:267–284PubMedGoogle Scholar
  19. 19.
    Bloom W, Fawcett DW (1975) A textbook of histology. WB Saunders, Philadelphia, pp 964–992Google Scholar
  20. 20.
    Arenberg IK, Norback DH, Shambaugh GE (1982) Ultrastructural analysis of endolymphatic sac biopsies: biopsy technique and identification of endolymphatic sac epithelium. Arch Otolaryngol 108:292–298PubMedGoogle Scholar
  21. 21.
    Bold EL, Wanamaker JR, Hughes GB, et al (1995) Adenomatous lesions of the temporal bone: immunohistochemical analysis and theories of histogenesis. Am J Otol 16:146–152PubMedGoogle Scholar
  22. 22.
    Zahtz GD, Zielinski B, Abramson AL (1981) Benign adenoma of the middle ear cavity causing facial paralysis. Otolaryngol Head Neck Surg 89:624–628PubMedGoogle Scholar
  23. 23.
    Batsakis JG (1989) Adenomatous tumors of the middle ear. Ann Otol Rhinol Laryngol 98:749–752PubMedGoogle Scholar
  24. 24.
    Polinsky MN, Brunberg JA, McKeever PE, et al (1994) Aggressive papillary middle ear tumors: a report of two cases with review of the literature. Neurosurgery 35:493–497PubMedCrossRefGoogle Scholar

Copyright information

© The Japan Society of Brain Tumor Pathology 1999

Authors and Affiliations

  • Kenichiroh Asano
    • 1
  • Tetsuji Sekiya
    • 1
  • Toru Hatayama
    • 1
  • Masahiko Tanaka
    • 1
  • Atsuhito Takemura
    • 1
  • Shigeharu Suzuki
    • 1
  • Osami Kubo
    • 2
  • Yoshihiro Ishihara
    • 3
  1. 1.Department of NeurosurgeryHirosaki University, School of MedicineHirosaki, Aomori PrefectureJapan
  2. 2.Department of Neurosurgery, Neurological InstituteTokyo Women's Medical CollegeTokyoJapan
  3. 3.Gerlatric Health Faculties Machida-SakuranboTokyoJapan

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