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Head and Neck Pathology

, Volume 12, Issue 3, pp 392–406 | Cite as

Ear and Temporal Bone Pathology: Neural, Sclerosing and Myofibroblastic Lesions

  • A. N. Flaman
  • J. K. Wasserman
  • D. H. Gravel
  • B. M. Purgina
Special Issue: Ear

Abstract

Neural, sclerosing, and myofibroblastic lesions of the ear and temporal bone present diagnostic challenges for both clinicians and pathologists due to significant overlap in their clinical presentations, histologic appearances, and immunohistochemical profiles. While some of these lesions, such as schwannomas, are relatively common, others are rendered even more difficult because they are encountered very rarely in routine surgical pathology practice. This review is intended to provide an update on the pathology of some of the most commonly encountered primary diagnostic entities for the ear and temporal bone, and includes the following neural lesions: schwannoma, meningioma, and encephalocele/meningocele. Sclerosing lesions that will be discussed include spindle cell and sclerosing rhabdomyosarcoma, sclerosing epithelioid fibrosarcoma, and sclerosing paraganglioma. Finally, myofibroblastic lesions that will be reviewed are nodular fasciitis, IgG4-related disease, and solitary fibrous tumor. For each of these lesions, the differential diagnosis and useful ancillary tests will be discussed in the context of a broad range of additional primary and secondary lesions.

Keywords

Temporal bone Ear Neurilemmoma Meningioma Encephalocele Rhabdomyosarcoma Solitary fibrous tumor Paraganglioma Fibrosarcoma 

Notes

Compliance with Ethical Standards

Conflict of interest

The authors have no conflicts of interest to disclose.

Ethical Approval

This article does not contain any studies with human participants performed by any of the authors.

References

  1. 1.
    Sandison A, Thompson LDR, Wenig BM. Tumours of the middle and inner ear: vestibular schwannoma. In: El-Naggar AK et al., editors. WHO classification of head and neck tumours. Lyon: IARC; 2017.Google Scholar
  2. 2.
    Bari ME, et al. Malignancy in a vestibular schwannoma. Report of a case with central neurofibromatosis, treated by both stereotactic radiosurgery and surgical excision, with a review of the literature. Br J Neurosurg. 2002;16(3):284–9.CrossRefPubMedGoogle Scholar
  3. 3.
    Carlson ML, et al. Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation. J Neurosurg. 2016;125(5):1120–9.CrossRefPubMedGoogle Scholar
  4. 4.
    Seferis C, et al. Malignant transformation in vestibular schwannoma: report of a single case, literature search, and debate. J Neurosurg. 2014;121(Suppl):160–6.PubMedGoogle Scholar
  5. 5.
    Nielsen GP, Antonescu CR, Lothe R. Malignant peripheral nerve sheath tumour. 4th ed. In: Fletcher CDM et al., editors. WHO classification of tumours of soft tissue and bone. Lyon: IARC; 2013.Google Scholar
  6. 6.
    Jo VY, Fletcher CD. Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases. Am J Surg Pathol. 2015;39(5):673–82.CrossRefPubMedGoogle Scholar
  7. 7.
    Prieto-Granada CN, et al. Loss of H3K27me3 expression is a highly sensitive marker for sporadic and radiation-induced MPNST. Am J Surg Pathol. 2016;40(4):479–89.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Schaefer IM, Fletcher CD, Hornick JL. Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics. Mod Pathol. 2016;29(1):4–13.CrossRefPubMedGoogle Scholar
  9. 9.
    Thompson LD, et al. Primary ear and temporal bone meningiomas: a clinicopathologic study of 36 cases with a review of the literature. Mod Pathol. 2003;16(3):236–45.CrossRefPubMedGoogle Scholar
  10. 10.
    Liu Y, et al. Primary extradural meningiomas in head: a report of 19 cases and review of literature. Int J Clin Exp Pathol. 2015;8(5):5624–32.PubMedPubMedCentralGoogle Scholar
  11. 11.
    Dixon J, Zammit-Maempel I, Hill J. External auditory canal meningioma: imaging features in a series of three cases. J Laryngol Otol. 2017;131(3):273–9.CrossRefPubMedGoogle Scholar
  12. 12.
    Mingo K, et al. Hyperostotic en plaque meningioma mimicking fibrous dysplasia of the temporal bone. Otol Neurotol. 2016;37(9):e317–8.CrossRefPubMedGoogle Scholar
  13. 13.
    Thompson LD. Ear and temporal bone meningioma. Ear Nose Throat J. 2016;95(4–5):146.PubMedGoogle Scholar
  14. 14.
    Ereno C, et al. Temporal bone secretory meningioma presenting as a middle ear mass. Pathol Res Pract. 2006;202(6):481–4.CrossRefPubMedGoogle Scholar
  15. 15.
    Perry A, et al., Meningioma. In: Louis DN et al., editor. WHO classification of tumours of the central nervous system. Lyon: IARC; 2016.Google Scholar
  16. 16.
    Hahn HP, Bundock EA, Hornick JL. Immunohistochemical staining for claudin-1 can help distinguish meningiomas from histologic mimics. Am J Clin Pathol. 2006;125(2):203–8.CrossRefPubMedGoogle Scholar
  17. 17.
    Pravdenkova S, et al. Progesterone and estrogen receptors: opposing prognostic indicators in meningiomas. J Neurosurg. 2006;105(2):163–73.CrossRefPubMedGoogle Scholar
  18. 18.
    Mawrin C, Perry A. Pathological classification and molecular genetics of meningiomas. J Neurooncol. 2010;99(3):379–91.CrossRefPubMedGoogle Scholar
  19. 19.
    Vallicioni JM, et al. Idiopathic temporal encephalocele: report of two cases. Am J Otol. 1999;20(3):390–3.PubMedGoogle Scholar
  20. 20.
    Gyure KA, Thompson LD, Morrison AL. A clinicopathological study of 15 patients with neuroglial heterotopias and encephaloceles of the middle ear and mastoid region. Laryngoscope. 2000;110(10 Pt 1):1731–5.CrossRefPubMedGoogle Scholar
  21. 21.
    Shim HJ, et al. Neuroglial choristoma of the middle ear with massive tympanosclerosis: a case report and literature review. J Audiol Otol. 2016;20(3):179–82.CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Turner JH, Richmon JD. Head and neck rhabdomyosarcoma: a critical analysis of population-based incidence and survival data. Otolaryngol Head Neck Surg. 2011;145(6):967–73.CrossRefPubMedGoogle Scholar
  23. 23.
    Owosho AA, et al. Clinical and molecular heterogeneity of head and neck spindle cell and sclerosing rhabdomyosarcoma. Oral Oncol. 2016;58:e6–e11.CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Nascimento AG, Barr F. Skeletal-muscle tumours: spindle cell/sclerosing rhabdomyosarcoma. 4th ed. In: WHO classification of tumours of soft tissue and bone. Lyon: IARC; 2013.Google Scholar
  25. 25.
    Rekhi B, Singhvi T. Histopathological, immunohistochemical and molecular cytogenetic analysis of 21 spindle cell/sclerosing rhabdomyosarcomas. APMIS. 2014;122(11):1144–52.PubMedGoogle Scholar
  26. 26.
    Folpe AL, et al. Sclerosing rhabdomyosarcoma in adults: report of four cases of a hyalinizing, matrix-rich variant of rhabdomyosarcoma that may be confused with osteosarcoma, chondrosarcoma, or angiosarcoma. Am J Surg Pathol. 2002;26(9):1175–83.CrossRefPubMedGoogle Scholar
  27. 27.
    Mentzel T, Katenkamp D. Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases. Virchows Arch. 2000;436(4):305–11.CrossRefPubMedGoogle Scholar
  28. 28.
    Mentzel T, Kuhnen C. Spindle cell rhabdomyosarcoma in adults: clinicopathological and immunohistochemical analysis of seven new cases. Virchows Arch. 2006;449(5):554–60.CrossRefPubMedGoogle Scholar
  29. 29.
    Agaram NP, et al. Recurrent MYOD1 mutations in pediatric and adult sclerosing and spindle cell rhabdomyosarcomas: evidence for a common pathogenesis. Genes Chromosomes Cancer. 2014;53(9):779–87.CrossRefPubMedPubMedCentralGoogle Scholar
  30. 30.
    Bishop JA, et al. Rhabdomyoblastic differentiation in head and neck malignancies other than rhabdomyosarcoma. Head Neck Pathol. 2015;9(4):507–18.CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Weissinger SE, et al. A diagnostic algorithm to distinguish desmoplastic from spindle cell melanoma. Mod Pathol. 2014;27(4):524–34.CrossRefPubMedGoogle Scholar
  32. 32.
    Ossendorf C, et al. Sclerosing epithelioid fibrosarcoma: case presentation and a systematic review. Clin Orthop Relat Res. 2008;466(6):1485–91.CrossRefPubMedPubMedCentralGoogle Scholar
  33. 33.
    Antonescu CR, et al. Sclerosing epithelioid fibrosarcoma: a study of 16 cases and confirmation of a clinicopathologically distinct tumor. Am J Surg Pathol. 2001;25(6):699–709.CrossRefPubMedGoogle Scholar
  34. 34.
    Folk GS, et al. Oral and maxillofacial sclerosing epithelioid fibrosarcoma: report of five cases. Head Neck Pathol. 2007;1(1):13–20.CrossRefPubMedPubMedCentralGoogle Scholar
  35. 35.
    Hasan Z, Clark JR, Fowler A. A facial dismasking approach for resection of an infratemporal fossa sclerosing epithelioid fibrosarcoma. ANZ J Surg. 2011;81(12):947–8.CrossRefPubMedGoogle Scholar
  36. 36.
    Doyle LA, et al. MUC4 is a sensitive and extremely useful marker for sclerosing epithelioid fibrosarcoma: association with FUS gene rearrangement. Am J Surg Pathol. 2012;36(10):1444–51.CrossRefPubMedGoogle Scholar
  37. 37.
    Mohamed M, Fisher C, Thway K. Low-grade fibromyxoid sarcoma: clinical, morphologic and genetic features. Ann Diagn Pathol. 2017;28:60–7.CrossRefPubMedGoogle Scholar
  38. 38.
    Doyle LA, et al. MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma. Am J Surg Pathol. 2011;35(5):733–41.CrossRefPubMedGoogle Scholar
  39. 39.
    Williams MD. Paragangliomas of the head and neck: an overview from diagnosis to genetics. Head Neck Pathol. 2017;11(3):278–87.CrossRefPubMedPubMedCentralGoogle Scholar
  40. 40.
    Williams MD, Tischler AS. Update from the 4th edition of the World Health Organization classification of head and neck tumours: paragangliomas. Head Neck Pathol. 2017;11(1):88–95.CrossRefPubMedPubMedCentralGoogle Scholar
  41. 41.
    Plaza JA, et al. Sclerosing paraganglioma: report of 19 cases of an unusual variant of neuroendocrine tumor that may be mistaken for an aggressive malignant neoplasm. Am J Surg Pathol. 2006;30(1):7–12.CrossRefPubMedGoogle Scholar
  42. 42.
    Weinreb I, et al. Nodular fasciitis of the head and neck region: a clinicopathologic description in a series of 30 cases. J Cutan Pathol. 2009;36(11):1168–73.CrossRefPubMedGoogle Scholar
  43. 43.
    Gibson TC, Bishop JA, Thompson LD. Parotid gland nodular fasciitis: a clinicopathologic series of 12 cases with a review of 18 cases from the literature. Head Neck Pathol. 2015;9(3):334–44.CrossRefPubMedGoogle Scholar
  44. 44.
    Erickson-Johnson MR, et al. Nodular fasciitis: a novel model of transient neoplasia induced by MYH9-USP6 gene fusion. Lab Invest. 2011;91(10):1427–33.CrossRefPubMedGoogle Scholar
  45. 45.
    Cowan ML, et al. Low-grade fibromyxoid sarcoma of the head and neck: a clinicopathologic series and review of the literature. Head Neck Pathol. 2016;10(2):161–6.CrossRefPubMedGoogle Scholar
  46. 46.
    Lau PP, et al. EWSR1-CREB3L1 gene fusion: a novel alternative molecular aberration of low-grade fibromyxoid sarcoma. Am J Surg Pathol. 2013;37(5):734–8.CrossRefPubMedGoogle Scholar
  47. 47.
    Devaney KO, et al. Inflammatory myofibroblastic tumors of the head and neck: evaluation of clinicopathologic and prognostic features. Eur Arch Otorhinolaryngol. 2012;269(12):2461–5.CrossRefPubMedGoogle Scholar
  48. 48.
    Bhatti RM, Stelow EB. IgG4-related disease of the head and neck. Adv Anat Pathol. 2013;20(1):10–6.CrossRefPubMedGoogle Scholar
  49. 49.
    Deshpande V. IgG4 related disease of the head and neck. Head Neck Pathol. 2015;9(1):24–31.CrossRefPubMedPubMedCentralGoogle Scholar
  50. 50.
    Mulholland GB, et al. Immunoglobulin G4-related diseases in the head and neck: a systematic review. J Otolaryngol Head Neck Surg. 2015;44:24.CrossRefPubMedPubMedCentralGoogle Scholar
  51. 51.
    Takano K, et al. Recent advances in knowledge regarding the head and neck manifestations of IgG4-related disease. Auris Nasus Larynx. 2017;44(1):7–17.CrossRefPubMedGoogle Scholar
  52. 52.
    Deshpande V, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181–92.CrossRefPubMedGoogle Scholar
  53. 53.
    He CY, et al. Inflammatory myofibroblastic tumors of the nasal cavity and paranasal sinus: a clinicopathologic study of 25 cases and review of the literature. Eur Arch Otorhinolaryngol. 2015;272(4):789–97.CrossRefPubMedGoogle Scholar
  54. 54.
    Ong HS, et al. Head and neck inflammatory myofibroblastic tumor (IMT): evaluation of clinicopathologic and prognostic features. Oral Oncol. 2012;48(2):141–8.CrossRefPubMedGoogle Scholar
  55. 55.
    Pierry C, et al. Polypoid laryngeal inflammatory myofibroblastic tumors: misleading lesions: description of six cases showing ALK overexpression. Am J Clin Pathol. 2015;144(3):511–6.CrossRefPubMedGoogle Scholar
  56. 56.
    Lazaridou M, et al. Inflammatory myofibroblastic tumour of the maxillary sinus and the oral cavity. Oral Maxillofac Surg. 2014;18(1):111–4.CrossRefPubMedGoogle Scholar
  57. 57.
    Rezk S, et al. Solitary fibrous tumor of the auditory canal. Arch Pathol Lab Med. 2004;128(12):e169–71.PubMedGoogle Scholar
  58. 58.
    Izumaru S, Yoshida Y, Nakashima T. A solitary fibrous tumor in the external auditory meatus. Auris Nasus Larynx. 2004;31(1):65–7.CrossRefPubMedGoogle Scholar
  59. 59.
    Lee CK, Lee HJ. Is a solitary fibrous tumor in the external auditory canal benign? J Audiol Otol. 2016;20(2):120–2.CrossRefPubMedPubMedCentralGoogle Scholar
  60. 60.
    Kunzel J, et al. Head and neck solitary fibrous tumors: a rare and challenging entity. Eur Arch Otorhinolaryngol. 2016;273(6):1589–98.CrossRefPubMedGoogle Scholar
  61. 61.
    Cox DP, Daniels T, Jordan RC. Solitary fibrous tumor of the head and neck. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2010;110(1):79–84.CrossRefPubMedGoogle Scholar
  62. 62.
    Doyle LA, et al. Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics. Mod Pathol. 2014;27(3):390–5.CrossRefPubMedGoogle Scholar
  63. 63.
    Yoshida A, et al. STAT6 immunohistochemistry is helpful in the diagnosis of solitary fibrous tumors. Am J Surg Pathol. 2014;38(4):552–9.CrossRefPubMedGoogle Scholar
  64. 64.
    Demicco EG, et al. Extensive survey of STAT6 expression in a large series of mesenchymal tumors. Am J Clin Pathol. 2015;143(5):672–82.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • A. N. Flaman
    • 1
  • J. K. Wasserman
    • 1
  • D. H. Gravel
    • 1
  • B. M. Purgina
    • 1
  1. 1.Division of Anatomical Pathology, Department of Pathology and Laboratory MedicineThe Ottawa Hospital/University of OttawaOttawaCanada

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