Abstract
Neuroendocrine neoplasms of the ear are uncommon primary tumors. There is a reported debate in the literature as to the true nature of these tumors and how to classify them. However, there is a kind of consensus that they can represent a spectrum of a common entity with dual glandular and neuroendocrine differentiation. The differential diagnosis of these tumors is broad but their morphologic, immunohistochemical, and radiographic features help to differentiate them from other entities. The tumors have mostly bland histology and indolent biological behavior, yet they may recur and metastasize. It is important to accurately recognize these tumors in order to perform the right management and limit their morbidity. In this article, we review the pathogenesis, histology, immunohistochemical features of neurornedocrine neoplasms of the ear and we discuss their differential diagnosis, clinical behavior and management.
Similar content being viewed by others
References
Agaimy A, Lell M, Schaller T, Märkl B, Hornung J. (2015). ‘Neuroendocrine’ middle ear adenomas: consistent expression of the transcription factor ISL1 further supports their neuroendocrine derivation. Histopathology, 66(1), 182–91. https://doi.org/10.1111/his.2015.66.issue-1.
Bahrami A, Gown AM, Baird GS, Hicks MJ, Folpe AL. Aberrant expression of epithelial and neuroendocrine markers in alveolar rhabdomyosarcoma: a potentially serious diagnostic pitfall. Mod Pathol. 2008;21(7):795–806. https://doi.org/10.1038/modpathol.2008.86.
Barnes L. Surgical pathology of the head and neck. New York: Informa Healthcare; 2009.
Bell D, El-Naggar AK, Gidley PW. Middle ear adenomatous neuroendocrine tumors: a 25-year experience at MD Anderson Cancer Center. Virchows Arch. 2017;471(5):667–72. https://doi.org/10.1007/s00428-017-2155-6.
Bierry G, Riehm S, Marcellin L, Stierlé JL, Veillon F. (2010). Middle ear adenomatous tumor: A not so rare glomus tympanicum-mimicking lesion. J Neuroradiol; 37(2), 116 – 21. https://doi.org/10.1016/j.neuad.2009.05.003.
Cardoso F, Monteiro EM, Lopes L, Avila M, Scarioli B. Adenomatous tumors of the middle ear: a literature review. Int Arch Otorhinol 2017;21(03):308–12. https://doi.org/10.1055/s-0037-1601400.
El Naggar AK, Chan JKC, Takata T, Slootweg PJ. WHO classification of the head and neck tumours. IARC Press; 2017.
Hyams VJ, Michaels L. (1976) Benign adenomatous neoplasm (adenoma) of the middle ear. Clin Otolaryngol Allied Sci, 1(1),17–26.
Murphy GF, Pilch BZ, Dickersin GR, Goodman ML, Nadol JR. (1980). Carcinoid tumor of the middle ear. Am J Clin Pathol,73 (6), 816–823.
Nader M, Bell D, Ginsberg L, Demonte F, Gunn GB, Gidley PW. (2017). The first reported case of primary intestinal-type adenocarcinoma of the middle ear and review of the literature. Otology Neurotol, https://doi.org/10.1097/mao.0000000000001541.
Pelosi S, Koss S. Adenomatous tumors of the middle ear. Otolaryngol Clin North Am. 2015;48(2):305–15. https://doi.org/10.1016/j.otc.2014.12.005.
Ramsey MJ, Nadol JR, Pilch BZ, McKenna MJ. Carcinoid tumor of the middle ear: Clinical features, recurrences, and metastases. Laryngoscope. 2005;115(9):1660–6.
Saliba I, Evrard A. Middle ear glandular neoplasms: adenoma, carcinoma or adenoma with neuroendocrine differentiation: a case series. Cases Journal. 2009. https://doi.org/10.1186/1757-1627-2-6508. 2 .
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(5):543–55. https://doi.org/10.1038/modpathol.3880561.
Wenig B. (2016). Atlas of Head and Neck Pathology. Amsterdam: Elsevier.
Acknowledgements
The author thanks Dr. Bin Xu for providing samples for images.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Katabi, N. Neuroendocrine Neoplasms of the Ear. Head and Neck Pathol 12, 362–366 (2018). https://doi.org/10.1007/s12105-018-0924-4
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12105-018-0924-4