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.

Download full-text


Available from: Lester D R Thompson
  • Source
    • "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]. "
    [Show abstract] [Hide abstract]
    ABSTRACT: . Despite modern radiological workup, surgeons can still be surprised by intraoperative findings or by the pathologist’s report. Materials & Methods . We describe the case of a 52-year-old male who was referred to our clinic with a single sided conductive hearing loss. He ultimately underwent middle ear exploration and excision of a middle ear tumour followed by second look and ossiculoplasty a year later. Results . Though preoperative CT and MRI scanning were suggestive of a congenital cholesteatoma, the pathologist’s report diagnosed a middle ear adenoma. Discussion . Middle ear glandular tumors are extremely rare and, despite numerous histological techniques, continue to defy satisfactory classification. Most surgeons advocate surgical excision though evidence of the tumour’s natural course and risk of recurrence is lacking.
    Full-text · Article · Jun 2014
  • Source
    • "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]. "
    [Show abstract] [Hide abstract]
    ABSTRACT: We herein report a 59-year-old male patient with a recurrent carcinoid tumor of the middle ear 7 years after a tympanomastoidectomy. The CT and dynamic MRI demonstrated an extensive tumor close to the carotid artery canal and the jugular bulb, and the tumor was removed by a partial petrosectomy with a transmastoid approach. The histopathological findings revealed a solid and trabecular tumor with cells positive for cytokeratin, chromogranin A, synaptophysin, and CD56. The MIB-1 antibody for the Ki-67 antigen was positive in 6.6% of the tumor cells. The relevant literature is reviewed in regard to the present case.
    Full-text · Article · Jun 2010 · International Journal of Otolaryngology
  • Source
    • "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. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Primary extracranial meningiomas are rare neoplasms, frequently misdiagnosed, resulting in inappropriate clinical management. To date, a large clinicopathologic study has not been reported. One hundred and forty-six cases diagnosed between 1970 and 1999 were retrieved from the files of the Armed Forces Institute of Pathology. Histologic features were reviewed, immunohistochemistry analysis was performed (n = 85), and patient follow-up was obtained (n = 110). The patients included 74 (50.7%) females and 72 (49.3%) males. Tumors of the skin were much more common in males than females (1.7:1). There was an overall mean age at presentation of 42.4 years, with a range of 0.3-88 years. The overall mean age at presentation was significantly younger for skin primaries (36.2 years) than for ear (50.1 years) and nasal cavity (47.1 years) primaries. Symptoms were in general non-specific and reflected the anatomic site of involvement, affecting the following areas in order of frequency: scalp skin (40.4%), ear and temporal bone (26%), and sinonasal tract (24%). The tumors ranged in size from 0.5 up to 8 cm, with a mean size of 2.3 cm. Histologically, the majority of tumors were meningothelial (77.4%), followed by atypical (7.5%), psammomatous (4.1%) and anaplastic (2.7%). Psammoma bodies were present in 45 tumors (30.8%), and bone invasion in 31 (21.2%) of tumors. The vast majority were WHO Grade I tumors (87.7%), followed by Grade II (9.6%) and Grade III (2.7%) tumors. Immunohistochemically, the tumor cells labeled for EMA (76%; 61/80), S-100 protein (19%; 15/78), CK 7 (22%; 12/55), and while there was ki-67 labeling in 27% (21/78), <3% of cells were positive. The differential diagnosis included a number of mesenchymal and epithelial tumors (paraganglioma, schwannoma, carcinoma, melanoma, neuroendocrine adenoma of the middle ear), depending on the anatomic site of involvement. Treatment and follow-up was available in 110 patients: Biopsy, local excision, or wide excision was employed. Follow-up time ranged from 1 month to 32 years, with an average of 14.5 years. Recurrences were noted in 26 (23.6%) patients, who were further managed by additional surgery. At last follow-up, recurrent disease was persistent in 15 patients (mean, 7.7 years): 13 patients were dead (died with disease) and two were alive; the remaining patients were disease free (alive 60, mean 19.0 years, dead 35, mean 9.6 years). There is no statistically significant difference in 5-year survival rates by site: ear and temporal bone: 83.3%; nasal cavity: 81.8%; scalp skin: 78.5%; other sites: 65.5% (P = 0.155). Meningiomas can present in a wide variety of sites, especially within the head and neck region. They behave as slow-growing neoplasms with a good prognosis, with longest survival associated with younger age, and complete resection. Awareness of this diagnosis in an unexpected location will help to avoid potential difficulties associated with the diagnosis and management of these tumors.
    Full-text · Article · Jun 2009 · Head and Neck Pathology
Show more