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Update From the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Tumours of the Ear

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Abstract

The 2017 fourth edition of the World Health Organization Classification of Tumours, specifically as it relates to the ear (Chap. 9), has several changes. Importantly, the number of entities has been significantly reduced by omitting tumors or lesions if they do not occur exclusively or predominantly at this site or if they are discussed in detail elsewhere in the book. These entities include: embryonal rhabdomyosarcoma, osteoma, exostosis, angiolymphoid hyperplasia with eosinophilia, Schneiderian papilloma, inverted papilloma, lipoma of the internal auditory canal, hemangioma, hematolymphoid tumors, and secondary tumors. Paraganglioma was included in the neck chapter. New entries include otosclerosis and cholesteatoma, while refinements to nomenclature, classification and criteria were incorporated into the ceruminous gland tumors and epithelial tumors of the middle and inner ear. Specifically, the middle and inner ear were combined, as practical limitations of origin and imaging make a definitive separation artificial. The classification reflects the state of current understanding for these uncommon entities, with this update only highlighting selected entities that were the most significantly changed.

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References

  1. Tumours of the Ear. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ, editors. WHO classification of tumours of the head and neck, 4th ed. Lyon: IARC Press; 2017;261–73.

  2. Sandison A, Thompson LDR. Tumours of the external auditory canal: squamous cell carcinoma. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ, editors. WHO classification of head and neck tumours, 4th ed. Lyon: IARC Press, 2017;263–64.

  3. Ouaz K, Robier A, Lescanne E, et al. Cancer of the external auditory canal. Eur Ann Otorhinolaryngol Head Neck Dis. 2013;130:175–82.

    Article  CAS  PubMed  Google Scholar 

  4. Johns ME, Headington JT. Squamous cell carcinoma of the external auditory canal. A clinicopathologic study of 20 cases. Arch Otolaryngol. 1974;100:45–9.

    Article  CAS  PubMed  Google Scholar 

  5. Wermker K, Kluwig J, Schipmann S, et al. Prediction score for lymph node metastasis from cutaneous squamous cell carcinoma of the external ear. Eur J Surg Oncol. 2015;41:128–35.

    Article  CAS  PubMed  Google Scholar 

  6. Shu MT, Lin HC, Lee JC, et al. Radiation-induced squamous cell carcinoma of the external auditory canal. Otol Neurotol. 2011;32:e24–e25.

    Article  PubMed  Google Scholar 

  7. Lo WC, Ting LL, Ko JY, et al. Malignancies of the ear in irradiated patients of nasopharyngeal carcinoma. Laryngoscope. 2008;118:2151–5.

    Article  PubMed  Google Scholar 

  8. Fleiner F, Jumah M, Goktas O. Cancer of the external auditory canal-diagnostic and treatment. Indian J Otolaryngol Head Neck Surg. 2009;61:270–4.

    Article  PubMed  Google Scholar 

  9. Miah MS, Crawford M, White SJ, et al. Malignant transformation from benign papillomatosis of the external auditory canal. Otol Neurotol. 2012;33:643–7.

    Article  PubMed  Google Scholar 

  10. Clark RR, Soutar DS, Hunter KD. A retrospective analysis of histological prognostic factors for the development of lymph node metastases from auricular squamous cell carcinoma. Histopathology. 2010;57:138–46.

    Article  PubMed  Google Scholar 

  11. Hosokawa S, Mizuta K, Takahashi G, et al. Carcinoma of the external auditory canal: histological and treatment groups. B-ENT. 2014;10:259–64.

    CAS  PubMed  Google Scholar 

  12. Repanos C, Mitchell D, Gandhi M, et al. Great auricular nerve perineural spread of squamous cell carcinoma. ANZ J Surg. 2012;82:179–80.

    Article  PubMed  Google Scholar 

  13. Mazzoni A, Danesi G, Zanoletti E. Primary squamous cell carcinoma of the external auditory canal: surgical treatment and long-term outcomes. Acta Otorhinolaryngol. 2014;34:129–37.

    CAS  Google Scholar 

  14. Stafford ND, Frootko NJ. Verrucous carcinoma in the external auditory canal. Am J Otol. 1986;7:443–5.

    CAS  PubMed  Google Scholar 

  15. Sandison A, Thompson LDR. Tumours of the external auditory canal: Ceruminous adenoma. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ. WHO classification of head and neck tumours, 4th ed. Lyon: IARC Press, 2017;265.

  16. Sandison A, Stenman G, Thompson LDR. Tumours of the external auditory canal: Ceruminous adenocarcinoma. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ. WHO Classification of Head and Neck Tumours, 4th ed. Lyon: IARC Press, 2017;264.

  17. Crain N, Nelson BL, Barnes EL, et al. Ceruminous gland carcinomas: a clinicopathologic and immunophenotypic study of 17 cases. Head Neck Pathol. 2009;3:1–17.

    Article  PubMed  Google Scholar 

  18. Dehner LP, Chen KT. Primary tumors of the external and middle ear. Benign and malignant glandular neoplasms. Arch Otolaryngol. 1980;106:13–9.

    Article  CAS  PubMed  Google Scholar 

  19. Mansour P, George MK, Pahor AL. Ceruminous gland tumours: a reappraisal. J Laryngol Otol. 1992;106:727–32.

    Article  CAS  PubMed  Google Scholar 

  20. Jan JC, Wang CP, Kwan PC, et al. Ceruminous adenocarcinoma with extensive parotid, cervical, and distant metastases: case report and review of literature. Arch Otolaryngol Head Neck Surg. 2008;134:663–6.

    Article  PubMed  Google Scholar 

  21. Perzin KH, Gullane P, Conley J. Adenoid cystic carcinoma involving the external auditory canal. A clinicopathologic study of 16 cases. Cancer. 1982;50:2873–83.

    Article  CAS  PubMed  Google Scholar 

  22. Lott Limbach AA, Hoschar AP, Thompson LD, et al. Middle ear adenomas stain for two cell populations and lack myoepithelial cell differentiation. Head Neck Pathol. 2012;6:345–53.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Ito K, Ito T, Tsukuda M, et al. An immunohistochemical study of adenoid cystic carcinoma of the external auditory canal. Eur Arch Otorhinolaryngol. 1993;250:240–4.

    Article  CAS  PubMed  Google Scholar 

  24. Thompson LD, Nelson BL, Barnes EL. Ceruminous adenomas: a clinicopathologic study of 41 cases with a review of the literature. Am J Surg Pathol. 2004;28:308–18.

    Article  PubMed  Google Scholar 

  25. Cankar V, Crowley H. Tumors Of ceruminous glands: a clinicopathological study Of 7 cases. Cancer. 1964;17:67–75.

    Article  CAS  PubMed  Google Scholar 

  26. Wetli CV, Pardo V, Millard M, et al. Tumors of ceruminous glands. Cancer. 1972;29:1169–78.

    Article  CAS  PubMed  Google Scholar 

  27. Iqbal A, Newman P. Ceruminous gland neoplasia. Br J Plast Surg. 1998;51:317–20.

    Article  CAS  PubMed  Google Scholar 

  28. Sandison A. Tumours of the middle and inner ear: aggressive papillary tumour. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ. WHO classification of head and neck tumours, 4th ed. Lyon: IARC Press, 2017;266–67.

  29. Duderstadt M, Forster C, Welkoborsky HJ, et al. Adenomatous tumors of the middle ear and temporal bone: clinical, morphological and tumor biological characteristics of challenging neoplastic lesions. Eur Arch Otorhinolaryngol. 2012;269:823–31.

    Article  CAS  PubMed  Google Scholar 

  30. Tysome JR, Harcourt J, Patel MC, et al. Aggressive papillary tumor of the middle ear: a true entity or an endolymphatic sac neoplasm? Ear Nose Throat J. 2008;87:378–93.

    PubMed  Google Scholar 

  31. Siedentop KH, Jeantet C. Primary adenocarcinoma of the middle ear. Report of three cases. Ann Otol Rhinol Laryngol. 1961;70:719–33.

    Article  CAS  PubMed  Google Scholar 

  32. Dadas B, Alkan S, Turgut S, et al. Primary papillary adenocarcinoma confined to the middle ear and mastoid. Eur Arch Otorhinolaryngol. 2001;258:93–5.

    Article  CAS  PubMed  Google Scholar 

  33. Polinsky MN, Brunberg JA, McKeever PE, et al. Aggressive papillary middle ear tumors: a report of two cases with review of the literature. Neurosurgery. 1994;35:493–7.

    Article  CAS  PubMed  Google Scholar 

  34. Gaffey MJ, Mills SE, Fechner RE, et al. Aggressive papillary middle-ear tumor. A clinicopathologic entity distinct from middle-ear adenoma. Am J Surg Pathol. 1988;12:790–7.

    Article  CAS  PubMed  Google Scholar 

  35. Goebel JA, Smith PG, Kemink JL, et al. Primary adenocarcinoma of the temporal bone mimicking paragangliomas: radiographic and clinical recognition. Otolaryngol Head Neck Surg. 1987;96:231–8.

    CAS  PubMed  Google Scholar 

  36. Muller M, Zammit-Maempel I, Hill J, et al. An unusual middle-ear mass. J Laryngol Otol. 2010;124:108–10.

    Article  CAS  PubMed  Google Scholar 

  37. Kawabata S, Hollander MC, Munasinghe JP, et al. Epidermal growth factor receptor as a novel molecular target for aggressive papillary tumors in the middle ear and temporal bone. Oncotarget. 2015;6:11357–68.

    Article  PubMed  PubMed Central  Google Scholar 

  38. Sandison A. Tumours of the middle and inner ear: endolymphatic sac tumour. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ. WHO classification of head and neck tumours, 4th ed. Lyon: IARC Press, 2017; 267–68.

  39. Michaels L. Origin of endolymphatic sac tumor. Head Neck Pathol. 2007;1:104–11.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Heffner DK. Low-grade adenocarcinoma of probable endolymphatic sac origin A clinicopathologic study of 20 cases. Cancer. 1989;64:2292–302.

    Article  CAS  PubMed  Google Scholar 

  41. Manski TJ, Heffner DK, Glenn GM, et al. Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel-Lindau disease. JAMA. 1997;277:1461–6.

    Article  CAS  PubMed  Google Scholar 

  42. Megerian CA, Haynes DS, Poe DS, et al. Hearing preservation surgery for small endolymphatic sac tumors in patients with von Hippel-Lindau syndrome. Otol Neurotol. 2002;23:378–87.

    Article  PubMed  Google Scholar 

  43. Kupferman ME, Bigelow DC, Carpentieri DF, et al. Endolymphatic sac tumor in a 4-year-old boy. Otol Neurotol. 2004;25:782–6.

    Article  PubMed  Google Scholar 

  44. Skalova A, Sima R, Bohus P, et al. Endolymphatic sac tumor (aggressive papillary tumor of middle ear and temporal bone): report of two cases with analysis of the VHL gene. Pathol Res Pract. 2008;204:599–606.

    Article  CAS  PubMed  Google Scholar 

  45. Lonser RR, Kim HJ, Butman JA, et al. Tumors of the endolymphatic sac in von Hippel-Lindau disease. NEngl J Med. 2004;350:2481–6.

    Article  CAS  Google Scholar 

  46. Sandison A. Tumours of the middle and inner ear: otosclerosis. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ. WHO classification of head and neck tumours, 4th ed. Lyon: IARC Press, 2017;268–69.

  47. Bittermann AJ, Wegner I, Noordman BJ, et al. An introduction of genetics in otosclerosis: a systematic review. Otolaryngol Head Neck Surg. 2014;150:34–9.

    Article  PubMed  Google Scholar 

  48. Karosi T, Sziklai I. Etiopathogenesis of otosclerosis. Eur Arch Otorhinolaryngol. 2010;267:1337–49.

    Article  PubMed  Google Scholar 

  49. Michaels L, Soucek S. Atypical mature bone in the otosclerotic otic capsule as the differentiated zone of an invasive osseous neoplasm. Acta Otolaryngol. 2014;134:118–23.

    Article  PubMed  Google Scholar 

  50. Rudic M, Keogh I, Wagner R, et al. The pathophysiology of otosclerosis: review of current research. Hear Res. 2015;330:51–6.

    Article  CAS  PubMed  Google Scholar 

  51. Declau F, van SM, Timmermans JP, et al. Prevalence of otosclerosis in an unselected series of temporal bones. Otol Neurotol. 2001;22:596–602.

    Article  CAS  PubMed  Google Scholar 

  52. Declau F, van SM, Timmermans JP, et al. Prevalence of histologic otosclerosis: an unbiased temporal bone study in Caucasians. Adv Otorhinolaryngol. 2007;65:6–16.

    CAS  PubMed  Google Scholar 

  53. Michaels L, Soucek S. Origin and growth of otosclerosis. Acta Otolaryngol. 2011;131:460–8.

    Article  PubMed  Google Scholar 

  54. Carvalho B, Hamerschmidt R, Telles JE, et al. Anatomopathology of the superstructure of the stapes in patients with otosclerosis. Int Arch Otorhinolaryngol. 2015;19:1–4.

    Article  PubMed  Google Scholar 

  55. Sandison A. Tumours of the middle and inner ear: cholesteatoma. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ. WHO classification of head and neck tumours, 4th ed. Lyon: IARC Press, 2017;269–70.

  56. Michaels L. Biology of cholesteatoma. Otolaryngol Clin N.Am. 1989;22:869–81.

    CAS  Google Scholar 

  57. Michaels L. Origin of congenital cholesteatoma from a normally occurring epidermoid rest in the developing middle ear. Int J Pediatr Otorhinolaryngol. 1988;15:51–65.

    Article  CAS  PubMed  Google Scholar 

  58. Kuo CL, Shiao AS, Yung M, et al. Updates and knowledge gaps in cholesteatoma research. Biomed Res Int. 2015;2015:854024.

    PubMed  PubMed Central  Google Scholar 

  59. Kuo CL. Etiopathogenesis of acquired cholesteatoma: prominent theories and recent advances in biomolecular research. Laryngoscope. 2015;125:234–40.

    Article  PubMed  Google Scholar 

  60. Nevoux J, Lenoir M, Roger G, et al. Childhood cholesteatoma. Eur Ann Otorhinolaryngol Head Neck Dis. 2010;127:143–50.

    Article  CAS  PubMed  Google Scholar 

  61. Louw L. Acquired cholesteatoma pathogenesis: stepwise explanations. J Laryngol Otol. 2010;124:587–93.

    Article  PubMed  Google Scholar 

  62. Jindal M, Riskalla A, Jiang D, et al. A systematic review of diffusion-weighted magnetic resonance imaging in the assessment of postoperative cholesteatoma. Otol Neurotol. 2011;32:1243–9.

    Article  PubMed  Google Scholar 

  63. Heilbrun ME, Salzman KL, Glastonbury CM, et al. External auditory canal cholesteatoma: clinical and imaging spectrum. AJNR Am J Neuroradiol. 2003;24:751–6.

    PubMed  Google Scholar 

  64. Wells MD, Michaels L. Mode of growth of acquired cholesteatoma. J Laryngol Otol. 1991;105:261–7.

    Article  CAS  PubMed  Google Scholar 

  65. Torske KR, Thompson LD. Adenoma versus carcinoid tumor of the middle ear: a study of 48 cases and review of the literature. Mod Pathol. 2002;15:543–55.

    Article  PubMed  Google Scholar 

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Correspondence to Lester D. R. Thompson.

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Special Issue: World Health Organization Classification Update

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Thompson, L.D.R. Update From the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Tumours of the Ear. Head and Neck Pathol 11, 78–87 (2017). https://doi.org/10.1007/s12105-017-0790-5

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