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

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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.

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References

  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. 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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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. Jo VY, Fletcher CD. Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases. Am J Surg Pathol. 2015;39(5):673–82.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    PubMed  PubMed Central  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  12. Mingo K, et al. Hyperostotic en plaque meningioma mimicking fibrous dysplasia of the temporal bone. Otol Neurotol. 2016;37(9):e317–8.

    Article  PubMed  Google Scholar 

  13. Thompson LD. Ear and temporal bone meningioma. Ear Nose Throat J. 2016;95(4–5):146.

    PubMed  Google Scholar 

  14. Ereno C, et al. Temporal bone secretory meningioma presenting as a middle ear mass. Pathol Res Pract. 2006;202(6):481–4.

    Article  PubMed  Google Scholar 

  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. 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.

    Article  PubMed  Google Scholar 

  17. Pravdenkova S, et al. Progesterone and estrogen receptors: opposing prognostic indicators in meningiomas. J Neurosurg. 2006;105(2):163–73.

    Article  PubMed  CAS  Google Scholar 

  18. Mawrin C, Perry A. Pathological classification and molecular genetics of meningiomas. J Neurooncol. 2010;99(3):379–91.

    Article  PubMed  CAS  Google Scholar 

  19. Vallicioni JM, et al. Idiopathic temporal encephalocele: report of two cases. Am J Otol. 1999;20(3):390–3.

    PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  23. Owosho AA, et al. Clinical and molecular heterogeneity of head and neck spindle cell and sclerosing rhabdomyosarcoma. Oral Oncol. 2016;58:e6–e11.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  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. Rekhi B, Singhvi T. Histopathological, immunohistochemical and molecular cytogenetic analysis of 21 spindle cell/sclerosing rhabdomyosarcomas. APMIS. 2014;122(11):1144–52.

    PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  27. Mentzel T, Katenkamp D. Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases. Virchows Arch. 2000;436(4):305–11.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  30. Bishop JA, et al. Rhabdomyoblastic differentiation in head and neck malignancies other than rhabdomyosarcoma. Head Neck Pathol. 2015;9(4):507–18.

    Article  PubMed  PubMed Central  Google Scholar 

  31. Weissinger SE, et al. A diagnostic algorithm to distinguish desmoplastic from spindle cell melanoma. Mod Pathol. 2014;27(4):524–34.

    Article  PubMed  CAS  Google Scholar 

  32. Ossendorf C, et al. Sclerosing epithelioid fibrosarcoma: case presentation and a systematic review. Clin Orthop Relat Res. 2008;466(6):1485–91.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  34. Folk GS, et al. Oral and maxillofacial sclerosing epithelioid fibrosarcoma: report of five cases. Head Neck Pathol. 2007;1(1):13–20.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  37. Mohamed M, Fisher C, Thway K. Low-grade fibromyxoid sarcoma: clinical, morphologic and genetic features. Ann Diagn Pathol. 2017;28:60–7.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  39. Williams MD. Paragangliomas of the head and neck: an overview from diagnosis to genetics. Head Neck Pathol. 2017;11(3):278–87.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  48. Bhatti RM, Stelow EB. IgG4-related disease of the head and neck. Adv Anat Pathol. 2013;20(1):10–6.

    Article  PubMed  CAS  Google Scholar 

  49. Deshpande V. IgG4 related disease of the head and neck. Head Neck Pathol. 2015;9(1):24–31.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  52. Deshpande V, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181–92.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  56. Lazaridou M, et al. Inflammatory myofibroblastic tumour of the maxillary sinus and the oral cavity. Oral Maxillofac Surg. 2014;18(1):111–4.

    Article  PubMed  Google Scholar 

  57. Rezk S, et al. Solitary fibrous tumor of the auditory canal. Arch Pathol Lab Med. 2004;128(12):e169–71.

    PubMed  Google Scholar 

  58. Izumaru S, Yoshida Y, Nakashima T. A solitary fibrous tumor in the external auditory meatus. Auris Nasus Larynx. 2004;31(1):65–7.

    Article  PubMed  Google Scholar 

  59. Lee CK, Lee HJ. Is a solitary fibrous tumor in the external auditory canal benign? J Audiol Otol. 2016;20(2):120–2.

    Article  PubMed  PubMed Central  Google Scholar 

  60. Kunzel J, et al. Head and neck solitary fibrous tumors: a rare and challenging entity. Eur Arch Otorhinolaryngol. 2016;273(6):1589–98.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  62. Doyle LA, et al. Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics. Mod Pathol. 2014;27(3):390–5.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

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Correspondence to B. M. Purgina.

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Flaman, A.N., Wasserman, J.K., Gravel, D.H. et al. Ear and Temporal Bone Pathology: Neural, Sclerosing and Myofibroblastic Lesions. Head and Neck Pathol 12, 392–406 (2018). https://doi.org/10.1007/s12105-018-0891-9

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  • DOI: https://doi.org/10.1007/s12105-018-0891-9

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