Adenoma versus Carcinoid Tumor of the Middle Ear: a Study of 48 Cases and Review of the Literature

Department of Endocrine and Otorhinolaryngic-Head and Neck Pathology, Armed Forces Institute of Pathology, Washington, D.C.
Modern Pathology (Impact Factor: 6.19). 06/2002; 15(5):543-55. DOI: 10.1038/modpathol.3880561
Source: PubMed


Carcinoid tumors and adenomas of the middle ear are rare neoplasms of indeterminate relationship to one another. Indeed, the literature is devoid of a large comprehensive series that evaluates the clinical, histologic, and immunophenotypic features of these tumors and their potential relationship. Forty-eight cases of middle ear adenoma between 1970 and 1995 were identified in the files of the Armed Forces Institute of Pathology. All cases were evaluated for cytomorphology and architectural pattern, in addition to their reactivity with various immunohistochemical reagents. Clinical follow-up was also obtained. A comprehensive review of the literature was performed with an eye toward correlating any distinct differences or similarities between carcinoid tumors and adenomas of the middle ear. The patients included 21 women and 27 men, aged 20 to 80 years (mean, 45.0 y). Patients experienced hearing loss, mass, and/or pain for a mean duration of 1.7 years. The mean tumor size was 0.8 cm, with six tumors extending beyond the middle ear. Histologically, the tumors were moderately cellular and unencapsulated, arranged in glandular, trabecular, and solid patterns composed of small cells with "salt and pepper" nuclear chromatin distribution. The tumor cells were immunoreactive with keratin, keratin 7, chromogranin, and human pancreatic polypeptide. All patients had surgery. No patients died with their disease (mean follow-up, 15.7 y). Eight patients developed recurrences that were treated surgically and were without evidence of disease at last follow-up (mean, 15.1 y). Our study and the review of the literature showed adenomas and carcinoid tumors of the middle ear to be essentially indistinguishable benign tumors. Middle ear adenoma most correctly describes their morphologic features and clinical behavior, although neuroendocrine adenoma of the middle ear may be a more accurate designation.

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    • "MEAs were first described by Hyams and Michaels in 1976 [1] and controversy exists as to what extent they represent a different entity from carcinoid tumours of the middle ear first described by Murphy et al. in 1980 [2]. Murphy felt that his case was better described as a carcinoid tumour because of ultrastructural evidence of a neuroendocrine differentiation. With only 94 published cases [3], current opinion leans towards classifying all these tumours as MEAs with a subset of neuroendocrine variants [4], as this appropriately implies their benign behavior. Some authors however argue that the appropriate terminology would be adenocarcinoid or amphicrine tumor in order to reflect its dual nature [5]. "
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    06/2014; 2014:342125. DOI:10.1155/2014/342125
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    • "They are strongly positive for cytokeratin, which is an epithelial tumor marker, and for neuroendocrinological markers such as chromogranin A, synaptophysin, CD56, and vimentin, but are negative for S-100. These tendencies are helpful for differentiating such tumors from paraganglioma, because paraganglioma is usually positive for S-100 [2] [16]. Most of the typical carcinoid tumors do not show a proliferative index detected by Ki-67 labeling using MIB-1 indices exceeding 10%, and most small-cell carcinomas show substantially higher values than 25% [17] [18] [19]. "
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    International Journal of Otolaryngology 06/2010; 2010:818673. DOI:10.1155/2010/818673
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    • "The differential diagnosis of extracranial meningiomas includes a variety of benign and malignant neoplasms dependent upon the anatomic site of involvement. Paraganglioma, schwannoma and metastatic carcinomas are the most frequent misdiagnoses for ear and temporal bone tumors [3, 19, 48]; carcinoma, melanoma, olfactory neuroblastoma, and aggressive psammomatoid ossifying fibroma for sinonasal tract lesions [6, 20, 49–51]; dermatofibroma, melanoma, fibrosarcoma, leiomyosarcoma, and synovial sarcoma for soft tissue and skin lesions, especially for Grade II or III tumors, and for the non-meningothelial types of meningiomas. "
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    Head and Neck Pathology 06/2009; 3(2):116-30. DOI:10.1007/s12105-009-0118-1
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