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Middle ear adenoma can be a rare cause of a middle ear mass. These benign tumors can have epithelial and/or neuroendocrine differentiation. In 1976, Hyams was the first to describe a series of these tumors. It is still thought that these tumors are indistinguishable in composition from each other. We report a case of middle ear adenoma, the clinical and pathologic findings of which illustrate the biological behavior of the middle ear. A 17-year-old male presented with a history of recurrent, bloody otorrhea and high-pitched tinnitus. We diagnosed him with congenital cholesteatoma and performed surgery to remove the entire tumor as well as the incus. However, on postoperative pathology examination, we finally diagnosed the tumor as middle-ear adenoma with neuroendocrine differentiation. In the two years following surgery, the patient's adenoma has not recurred, although long-term observation will be required.

Middle ear adenoma is a rare disease derived from the middle ear mucosa. It is usually lack specific findings and easily mistaken for other conditions, delaying a correct diagnosis. In particular, few cases with facial nerve paralysis have been reported. We describe a case of middle ear adenoma that caused hearing loss and recurrent facial paralysis in a 29-year-old woman. In an attempt to treat the facial nerve paralysis, we performed tympanomastoidectomy and facial nerve decompression. By pathological examination, we finally diagnosed it middle ear adenoma with neuroendocrine differentiation. Retrospectively, if we meet the antibiotic resistant mass in the middle ear, we should suspect the tumor. One year after surgery, the adenoma has not recurred but long-term observation is required.

MIDDLE EAR ADENOMA/MIDDLE EAR CARCINOID - AN UNPROBLEMATIC TUMOR?OBJECTIVES: Adenomas/carcinoid tumors of the middle ear are very rare tumors of young to middle aged patients. The terms adenoma and carcinoid tumor of the middle ear can be used as synonyms, with the WHO favouring the term middle ear adenoma (MEA). These tumors usually present with unspecific clinical symptoms and a long case history. They are classified as benign tumors with only very few reported cases of regional metastasis after years of disease. According to recent literature, the clinical course is usually uncomplicated with complete surgical excision being adequate therapy. METHODS AND PATIENTS: This study describes the clinical course and the diagnostic challenges in four cases of this rare tumor entity. RESULTS: The selected patients (2 males, 2 females, 25-38 years old) showed very similar clinical findings with decreased hearing acuity, tinnitus and sometimes pain. After the primary surgical excision up to 10 further operations were necessary, this being in contrast to the usual clinical course as described in the literature. In two cases a tumor recurrence was documented with one case recurring six times. In this case adjuvant radiotherapy (70 Gy) was performed. The histological differential diagnosis can also be problematic; in one case with a highly atypical morphology it was impossible to arrive at a definite diagnosis during the analysis of a frozen section. CONCLUSION: Adenomas of the middle ear can have a much more complicated clinical course than is suggested by the recent literature. The presented cases in this study and the analysis of previously published cases shows that the typical progression described by the current WHO-classification with unproblematic surgical management of the tumor and an uncomplicated further clinical course does not always correspond to reality. The main reason for this is the difficulty in obtaining a complete surgical excision. Therefore, a well-planned and comprehensive surgical management with a high frequency of follow-up examinations should be chosen. In complicated individual cases adjuvant radiotherapy can be helpful.

Middle ear adenoma (MEA) is a distinctive, rare entity that appears to be derived from the epithelium of the middle ear mucosa.A 39-year-old woman came to Mitsui Memorial Hospital, complaining of right tinnitus and ear fullness. Her right tympanic membrane was swollen by a middle ear mass lesion, and imaging studies such as CT and MRI of the temporal bone demonstrated a middle ear tumorous lesion that extend expansively. She did not feel otalgia or vertigo, and there was no otorrhea, facial palsy, nor facial spasm. Her right audiogram showed slight hearing loss (Pure tone average: 25.0dBHL) and her right tympanogram was Type B or C1. Thus, we performed a right ear operation. At surgery, a soft yellowish tumor was found in the middle ear from the promontory, which adhered closely to the tympanic membrane, but had not destroyed the ossicles or tympanic membrane. The facial canal was intact. The tumor was removed without complication. A frozen-section diagnosis could not exclude the possibility of carcinoma, but the symptoms and signs strongly suggested a benign tumor, so we performed tumor removal and Type III tympnoplasty. The histological features and immunochemical study of the tumor showed neuroendocrine differentiation with no sign of malignancy. The postoperative course was uneventful, with no evidence of recurrence 17 months after surgery.

Neuroendocrine adenoma of the middle ear has been described in literature as middle ear adenoma with neuroendocrine differentiation or carcinoid tumor. While there have been several case reports describing imaging features of carcinoid tumors of the ear and middle ear adenomas, in our literature review, we have not found a single case where bone formation is described as a prominent radiological feature. We report a first documented case of middle ear carcinoid tumor with new bone formation demonstrated on CT imaging and performed a review literature regarding the tumor. A differential diagnosis of neuroendocrine adenoma of the middle ear should be considered when there is prominent bone formation with a soft tissue mass in the middle ear on CT imaging.

Objective: Present four new cases of carcinoid tumor of the middle ear, two of which developed late recurrences and regional metastases. Review the literature to identify the clinical features, rate of recurrence, and incidence of metastasis of carcinoid tumor of the middle ear. Study Design: Retrospective chart review. Setting: Tertiary referral hospital. Patients: Eligibility criteria consist of a diagnosis of carcinoid tumor of middle ear. Intervention: Surgical excision of primary and metastatic disease. Main Outcome Measure: Clinical characteristics, rate of recurrence, and incidence of metastasis of carcinoid tumor of the middle ear. Results: Forty-six patients with carcinoid tumor of the middle ear are included in this report, 42 patients were identified from a review of the literature, and 4 new patients are presented. The most common presenting symptom was hearing loss. Surgical excision was the treatment with radical mastoidectomy being the most common procedure. Ten (22%) patients developed locally recurrent disease, and four (9%) developed regional metastases. Conclusions: Carcinoid tumor of the middle ear is an infrequent cause of a middle ear mass, with only 46 cases published. Despite previous assertions of benignancy, the findings of this study suggest that carcinoid tumor of the middle ear is indeed a potential low-grade malignancy with documented metastatic potential. Almost all middle ear adenomatous tumors (“adenoma” and “carcinoid”) show evidence of neuroendocrine differentiation, and so at least some middle ear carcinoids (“adenomas”) appear to represent well-differentiated neuroendocrine carcinomas. Presentation and symptoms are consistent with a middle ear mass and rarely include carcinoid syndrome. Surgical treatment is recommended and tailored to the extent of disease. Patients with carcinoid tumor of the middle ear require indefinite follow-up for possible recurrence or metastasis.

Background: Ceruminous gland neoplasms are rare neoplasms. To date, a large clinicopathologic study of benign ceruminous gland neoplasms has not been reported. Design: Forty-one cases of ceruminous gland adenomas diagnosed between 1970 and 2000 were retrieved from the files of the Armed Forces Institute of Pathology. Histologic features were reviewed, immunohistochemical analysis was performed (n = 21), and patient follow-up was obtained (n = 40). Results: The patients included 22 men and 19 women, 24 to 85 years of age (mean, 54.2 years). Patients presented clinically with a painless mass of the outer half of the external auditory canal (n = 33) or with hearing changes (n = 11). Symptoms were present for an average of 16.3 months. The polypoid masses affected the external auditory canal only and ranged in size from 0.4 to 2 cm in greatest dimension (mean, 1.1 cm). Histologically, the tumors demonstrated glands and small cysts lined by a tubuloglandular proliferation of inner ceruminous cells (cerumen-secreting epithelium with decapitation secretion) subtended by a spindled to cuboidal myoepithelial layer. A hyalinized stroma created an infiltrative pattern of growth; surface involvement (n = 8) was seen. Tumors were divided into ceruminous adenoma (n = 36), ceruminous pleomorphic adenoma (n = 4), and syringocystadenoma papilliferum (n = 1) types. The luminal cells were strongly and diffusely immunoreactive with CK7, while the basal cells were highlighted with CK5/6, S-100 protein, and p63. CDI 17 highlighted the luminal cells preferentially. The proliferation markers revealed a low index. Adenocarcinoma and middle ear adenoma are the principal differential consideration. Surgical excision was used in all patients. Four patients developed a recurrence due to incomplete excision. All patients were without evidence of disease at the last follow-up: alive (n = 28, mean 16.3 years) or dead (n = 12, mean 11.8 years). Conclusion: Ceruminous gland adenomas are the most common external auditory canal tumors. They demonstrate a dual cell population of basal myoepithelial-type cells and luminal ceruminous (ceruminal) cells. Cerumen pigment, CK7, and p63 can help to distinguish this tumor from other neoplasms that occur in the region. Complete surgical excision results in an excellent long-term clinical outcome.

We report a case of recurring middle ear adenoma with bone formation that was histologically difficult to differentiate from adenocarcinoma. The patient was a 31-year-old female. Preoperative clinical and CT findings in the 31-years-old woman examined suggested benign tumor of the middle ear. Although intraoperative pathological examination indicated adenocarcinoma, tympanoplasty in canal reconstruction was done. Malignancy was not considered because the tumor had not increased in size for 1 year and no bone erosion had occurred in the ossicles or middle ear cleft. The definitive pathological diagnosis was middle ear adenoma. The tumor repeatedly recurred thereafter, closely resembling normal middle ear mucosa and making it difficult to remove completely. When middle ear adenoma is suspected, surgical procedures should be determined carefully based on radiological and intraoperative findings in tumor expansion.

Middle ear adenoma (MEA) is a rare benign tumour. Here we report a case of MEA in a 39-year-old man with hearing loss. Some significant histopathological features of MEA and neuroendocrine differentiation identified by immunohistochemistry are described. The tumour showed remarkable and rare degenerative histological characteristics. There were a few tumour cells which were covered by degenerated tissue. Consequently, following frozen biopsy, the tumour was misdiagnosed as chronic ear inflammation. No recurrence was seen in this patient after 45 months of follow-up; regular clinical and radiological follow-up is necessary since recurrence is possible. A literature review for MEA is presented and this type of tumour is discussed clinically, microscopically and immunohistochemically. The differential diagnosis and associated treatment are described.

Middle ear adenoma is a rare disease that is thought to originate in the middle ear mucosa. It occurs over a wide age range, has no gender predilection, and is not characterized by specific symptoms or findings. The most frequent complaints are unilateral hearing loss and ear fullness. We report a 48-year-old woman with middle ear adenoma who had a history of unilateral ear fullness and hearing loss on the left side. Middle ear adenoma was suspected following a biopsy performed under local anesthesia. To remove the tumor, the patient underwent a left postauricular canal wall-up tympanoplasty type IIIc. Microscopic examination and immunohistochemistry confirmed a middle ear adenoma. In this case, we diagnosed the lesion as middle ear adenoma with neuroendocrine differentiation on the basis of the pathological findings. The patient has shown no recurrence for almost 5 years, but since this adenoma showed neuroendocrine differentiation, long-term observation is required.

As knowledge and understanding in pathology evolve, classifications and nomenclature also change to reflect those advances. The 2005 World Health Organization Classification of Head and Neck Tumours was a significant step towards diagnostic standardization of head and neck neuroendocrine carcinomas; however, in the last 10 years there have been new data supporting the recognition of “large cell neuroendocrine carcinoma” as a distinctive high grade carcinoma in the head and neck, a lesion not included in the 2005 Classification. In addition, the terms “middle ear adenoma” and “carcinoid tumor of middle ear” are still widely used to describe a neoplasm that is neither a pure adenoma nor a carcinoid tumor but a lesion with variable mixed exocrine and endocrine differentiation. Largely using the diagnostic criteria of the WHO classification of neuroendocrine carcinomas of the lung, we propose the terms “neuroendocrine carcinoma, grade 1”; “neuroendocrine carcinoma, grade 2”; “neuroendocrine carcinoma, grade 3, large cell type”; and “neuroendocrine carcinoma, grade 3, small cell type” for the classification of neuroendocrine carcinomas of the head and neck in a future WHO classification. In addition, we also proposed the term “mixed epithelial neuroendocrine tumor” of the middle ear as an alternative for “middle ear adenoma” and “carcinoid tumor of the middle ear”.

Adenomas of the middle ear are rare benign glandular neoplasms arising from the middle ear mucosa. After previous operations 25 and 15 years before, a 67-year-old female complained about dizziness, tinnitus, and unilateral hearing loss on the left side. A tumor in the tympanum that was revealed by otoscopy could be removed completely. Histological examinations showed an adenoma of the middle ear with cholesteatoma. This was inconsistent with the histological result of the operation in 1983, which had described a hidradenoma. An exact analysis of the preparations confirmed that a middle ear adenoma had already been present in 1983. Hidradenoma is one of the most important differential diagnoses. The characteristic histological sign of middle ear adenomas in contrast to hidradenomas is the lack of myoepithelial cells. In addition, it is very difficult to differentiate middle ear adenoma and adenocarcinoma using histopathological and clinical methods. Therefore, thorough follow-up is mandatory for patients after surgical treatment of middle ear adenomas.

The lining of the middle ear cleft is normally a thin modified respiratory type mucosa. Normal mucosa of the middle ear is devoid of salivary type seromucous glands. Middle ear adenoma is a rare tumor that seems to be derived from the middle ear mucosa. This tumor has been previously described under a variety of names because of its different nature and biological behavior. We herein report a case of middle ear tumor that shows adenomatous and neuroendocrine features. A 64-year-old woman presented with a history of hearing loss, tinnitus and stuffy feeling of the right ear. The patient was treated two times for ear polyp. She finally underwent a tympanomastoidectomy and there was no evidence of recurrence 18 months after the procedure. Histopathological examination displayed cuboidal and columnar cells, arranged as glands, trabeculae and solid sheets. Neuroendocrine differentiation was revealed by immunohistochemical staining with polyclonal antibodies against neuron specific enolase and chromogranin antigens.

✓ Middle ear adenocarcinoma is a very rare, locally invasive neoplasm assumed to arise from the middle ear mucosa. Although endolymphatic sac tumor (aggressive papillary middle ear tumor) and jugulotympanic paraganglioma may show brain invasion, intracranial extension of histologically confirmed middle ear adenocarcinoma has not been previously reported. The authors describe a 53-year-old man who suffered from otalgia and tinnitus for more than 10 years and from neurological deficits for 1 year due to a large temporal bone tumor that invaded the temporal lobe. A combined neurosurgical and otolaryngological resection was performed. Pathological analysis revealed a low-grade adenocarcinoma of a mixed epithelial—neuroendocrine phenotype, which showed a close histological similarity to, and topographical relationship with, middle ear epithelium. The authors conclude that middle ear adenocarcinoma belongs to the spectrum of extracranial tumors that have possible local extension to the brain.

Background Neuroendocrine tumors of the head and neck region may present problems in diagnosis. Middle ear carcinoid is a rare, recently recognized tumor, which to date has not been reported to metastasize. Methods We report the case of a 64-year-old man with a 9-year history of recurrent middle ear neoplasm and ipsilateral cervical lymphadenopathy. A microscopic parathyroid tumor was also identified. The approach to the diagnosis of this unusual combination is presented. Results The patient had a neuroendocrine tumor metastatic to multiple unilateral cervical lymph nodes, which was morphologically identical to his recurrent middle ear neoplasm. The neoplasm had the morphologic, immunohistochemical, and ultrastructural features of a carcinoid tumor. Conclusions This case illustrates that middle ear carcinoids may metastasize. We suggest that immunohistochemical studies be performed on all biopsy specimens from neoplasms of the middle ear, as distinction from the more common paraganglioma may be difficult on morphologic grounds alone. © 1999 John Wiley & Sons, Inc. Head Neck 21: 72–77, 1999.

Summary A primary adenomatous tumor of the middle ear was examined by light microscopy, ultrastructural and immunohistochemical techniques. In support of its extensive neuroendocrine differentiation, was the diffuse detection of neuron-specific enolase (NSE) and positive immunoreaction with antibodies to chromogranin and synaptophysin. The great majority of tumor cells contained neurosecretory granules and intraluminal mucin production could be focally detected. These characteristics confirm the diagnosis of a middle ear adenomatous tumor (MEAT) of a biphasic nature and with a prominent neuroendocrine component.

Middle ear adenoma is a benign tumor of the middle ear that can have exocrine (mucinous) and/or neuroendocrine differentiation. Early authors described a separate tumor with predominantly neuroendocrine differentiation as a middle ear carcinoid tumor, but these are now known to be the same tumor. We review the literature of this tumor, including the clinical presentation, gross pathology, histopathology, immunohistochemistry, differential diagnosis, and prognosis.

Middle-ear adenomas (MEAs) are rare neoplasms which can display several histological patterns and represent a diagnostic challenge. We present two cases of MEA which share some histopathological features such as medium to small cells forming solid infiltrating tumour nests as well as scattered glandular structures with Alcian blue and PAS positive material within. The second case also displayed a distinct and predominant "plasmacytoid' appearance which, in a small biopsy, might have been misleading. Both cases expressed an admixture of epithelial and neuroendocrine immunohistochemical markers, whereas ultrastructural study demonstrated electron dense granules. Taken together, these observations support a mixed epithelial and neuroendocrine nature for these neoplasms, the differential diagnosis of which includes paragangliomas and other tumours or tumour-like lesions involving less frequently the middle ear, such as meningiomas, plasmacytomas and inflammatory polyps. The existence of MEAs with plasmacytoid features should be remembered to avoid confusion with plasmacytomas, plasmacytoid myoepithelioma and plasma cell inflammatory infiltrates.

Summary Introduction: Adenomas with neuroendocrine differentiation are defined as neuroendocrine neoplasms, and they are rarely found in the head and neck. Objective: To describe two cases of a middle ear adenoma with neuroendocrine differentiation, with a literature review. Case Report: Patient 1 was a 41-year-old woman who presented with a 3-year history of left aural fullness associated with ipsilateral “hammer beating” tinnitus. Patient 2 was a 41-year-old male who presented with unilateral conductive hearing loss. Conclusion: Adenoma with neuroendocrine differentiation of the middle ear is a rare entity, but it should be considered in patients with tinnitus, aural fullness, and a retrotympanic mass and remembered as a diferential diagnosis of tympanic paraganglioma.

Middle ear adenomas (MEAs) are benign neoplasms along a spectrum with neuroendocrine neoplasms (carcinoid tumors). Immunohistochemical (IHC) staining for myoepithelial markers has not been reported in these tumors. The archives of the Cleveland Clinic, University of Virginia and Armed Forces Institute of Pathology were retrospectively searched for tumors arising within the middle ear with material available for IHC staining. Twelve cases of MEAs, four cases of jugulotympanic paragangliomas (JPGs), 10 cases of ceruminous adenomas (CAs) and four cases of ceruminous adenocarcinomas (CACs) were obtained. IHC staining was performed for smooth muscle actin (SMA), p63, S-100 protein, cytokeratin 5/6 (CK5/6), and cytokeratin 7 (CK7). The MEAs were positive for: CK7 (92 %, luminal), CK5/6 (92 %, abluminal), p63 (83 %, abluminal), and negative for SMA and S-100 protein. The JPGs were negative for CK7, CK5/6, p63 and SMA; S-100 protein highlighted sustentacular cells. The CAs were positive for: CK7 (100 %, luminal), CK5/6 (100 %, abluminal), S-100 protein (80 %, abluminal), p63 (100 %, abluminal), and SMA (90 %, abluminal). CACs demonstrated two patterns, (1) adenoid cystic carcinoma-type: positive for CK7 (100 %, luminal), CK5/6, S-100 protein, p63, and SMA (all 100 %, abluminal); and (2) conventional-type: CK7 (50 % luminal), and no CK5/6, SMA, S-100 protein, or p63 expression. The IHC profile of MEAs suggests that these tumors harbor at least two cell populations, including luminal and basal cells. However, unlike ceruminous adenomas, MEAs lack true myoepithelial differentiation given the absence of S-100 protein and SMA staining in all cases.

Adenomas of the middle ear are quite rare tumours of the ear and of recent recognition. Two cases of adenomatous neoplasms confined to the middle ear are presented. Usually these tumours are located on the promontorium or in the hypotympanon. The unique feature of the first described case is based on its origin in the epitympanic pouch of the tympanic membrane. The second case originated from the mucosa in the hypotympanon. The third case is a tumour-like lesion which was not distinguishable on clinical grounds from a jugulo-tympanic paraganglioma or a middle ear adenoma. The adenomas of the middle ear are histopathologically characterised by differentiated glandular structures within fibrous tissue and recurrences are rarely observed. Surgical removal by the endaural route gives an excellent prognosis as shown by the literature and our own clinical cases.

Adenomatous tumors of the middle ear and temporal bone are rare tumors. In this retrospective study, we examined nine patients who underwent surgery for an adenomatous tumor of the middle ear, mastoid cavity or eustachian tube. In seven patients, a middle ear adenoma (MEA) and in two patients an aggressive papillary tumor (APT) was diagnosed. We report the clinical, radiologic, morphologic, immunohistochemical and DNA image cytometrical characteristics that can help to correctly classify these tumors. Therapy consisted of surgical excision of the tumors in eight cases. In one elderly patient, only a large biopsy was taken, because this patient suffered from cardial and kidney disorders and was not suitable for an extended surgical approach. This patient received stereotactic radiotherapy. Seven patients underwent planned second look operation. Recurrences occurred in three patients (one with APT, two with MEA), whereas in two of these cases rather a residual tumor due to initial incomplete tumor resection occurred. By image analysis, DNA cytometry MEA were considered benign, whereas the appearance of aneuploid tumor cells in APT confirmed these tumors as low grade malignant lesions. The proliferation rates were equally low in both entities. APT and MEA are tumor entities which can only be correctly classified by a synopsis of histopathology, immunohistochemistry and DNA image cytometry. The recommended therapy is the complete tumor excision. In cases of APT, von Hippel-Lindau syndrome has to be excluded.

Adenomas and adenocarcinomas originating from the middle ear are few in number and have met with significant controversy in the literature. Our experience with 11 previously described patients is updated and five new cases are added. These tumors were seen in persons whose ages ranged from 7 to 77 years. The most common initial symptom was decreased hearing (11 cases), followed by otorrhea (4 cases), and otalgia (4 cases). At the time of diagnosis, facial nerve weakness was present in several patients (seven), and this was a poor prognostic sign. These glandular tumors engender controversy regarding histologic origin and the pathologic demarcation between adenoma and adenocarcinoma. In this review, we show that otorrhea, bone erosion, facial nerve involvement, and aggressive clinical behavior can be the result of lesions that appear microscopically benign. Furthermore, we present evidence of the similarity of these glandular lesions to paragangliomas and carcinoid tumors. Finally, pathologic data are presented to develop the previously unreported theory that the origin for this group of tumors is paraganglionic tissue.

The head and neck region contains a number of disparate organs ranging from skin to brain, and many of the neuroendocrine tumors of these organs have been discussed elsewhere in this book (skin, thyroid, and pituitary gland). Extra-adrenal paragangliomas, although also discussed elsewhere, will be mentioned and discussed briefly as they pertain to the ear and upper aerodigestive tract. This chapter will concentrate on neuroendocrine tumors of the ear, upper aerodigestive tract, and salivary glands. Focusing on these entities leaves only a few head- and neck-specific tumors to discuss, including middle ear adenoma, neuroendocrine carcinomas of the upper aerodigestive tract and salivary glands, uncommon and unique neuroectodermal tumors of the area, and, as mentioned, paraganglioma. Even if taken together, these tumors are exceedingly rare and little is known regarding the genetics of the tumors. While this limits our discussion, it does leave open some exciting avenues of possible research.

Middle ear adenomas are rare benign tumors, which can easily be mistaken for other conditions radiologically. They derive from the middle ear mucosa. We report the case of a 48-year-old man with a history of decreased left-sided hearing and intermittent pulsatile tinnitus. High-resolution CT of the temporal bones revealed a well-defined left middle ear soft-tissue attenuation abutting the head of the malleus. Surgical excision revealed a middle ear adenoma.

Carcinoid tumors of the middle ear are rare lesions that represent a class within the spectrum of adenomatous neoplasms. We present four cases that were diagnosed and treated at our institution since 1973, and review the pool of 12 cases in regard to their presentation, symptomatology, physical examination, audiometric and radiographic evaluations, operative procedure and findings, histopathology, and postoperative course. Successful treatment of these low-grade malignancies requires complete excision of the tumor mass, along with the ossicles if they are involved with disease, in order to prevent local recurrence. Although locally invasive, these tumors have a low propensity for distant metastasis. The diagnosis of carcinoid tumor should be considered in all cases of adenomatous neoplasms of the middle ear and mastoid.

Carcinoid tumor of the middle ear is an extremely rare lesion of the middle ear, and thus its diagnosis is frequently delayed. Some authors found it hard to differentiate middle ear carcinoid front the middle car adenoma. However, the balance of opinion is currently oil the side of considering it as a separate clinical entity. Definitive diagnosis is made by identifying neurosecretory tumor cells using immunohistochemistry and electron microscopy. It usually follows a nonaggressive clinical course, rarely metastases, and infrequently recurs after radical excisions. We present a patient with middle ear carcinoid, who is free of disease 2 years after the resection of the tumor mass with canal walldown procedure. The relevant literature is also herewith reviewed. (C) 2008 Elsevier Inc. All rights reserved.

Middle-ear ceruminous adenomas are rare benign neoplasms arising from the epithelium of the middle ear. Progressive hearing loss, ear fullness and tinnitus are common symptoms of this tumour; facial nerve paresis and vestibular disturbances occur very infrequently. We present two cases of middle-ear ceruminous adenomas, one showed rapid unilateral hearing loss with aural fullness, followed by purulent aural discharge and vertigo. In the second case, the disease affected an already deaf ear and the only symptom of the disease was increasing vertigo. The clinical features, intraoperative findings, and histological and radiological findings are presented. The cases are compared to those described in the literature.

"Primary" ear and temporal bone meningiomas are tumors that are frequently misdiagnosed and unrecognized, resulting in inappropriate clinical management. To date, a large clinicopathologic study of meningiomas in this anatomic site has not been reported. Thirty-six cases of ear and temporal bone meningiomas diagnosed between 1970 and 1996 were retrieved from our files. Histologic features were reviewed, immunohistochemical analysis was performed (n = 19), and patient follow-up was obtained (n = 35). The patients included 24 females and 12 males, aged 10-80 years (mean, 49.6 years), with female patients presenting at an older age (mean, 52.0 years) than male patients (mean, 44.8 years). Patients presented clinically with hearing changes (n = 20), otitis (n = 7), pain (n = 5), and/or dizziness/vertigo (n = 3). Symptoms were present for an average of 24.6 months. The tumors affected the middle ear (n = 25), external auditory canal (n = 4), or a combination of temporal bone and middle ear (n = 7). The tumors ranged in size from 0.5 to 4.5 cm in greatest dimension (mean, 1.2 cm). Radiographic studies demonstrated a central nervous system connection in 2 patients. Histologically, the tumors demonstrated features similar to those of intracranial meningiomas, including meningothelial (n = 33), psammomatous (n = 2), and atypical (n = 1). An associated cholesteatoma was identified in 9 cases. Immunohistochemical studies confirmed the diagnosis of meningioma with positive reactions for epithelial membrane antigen (79%) and vimentin (100%). The differential diagnosis includes paraganglioma, schwannoma, carcinoma, melanoma, and middle ear adenoma. Surgical excision was used in all patients. Ten patients developed a recurrence from 5 months to 2 years later. Five patients died with recurrent disease (mean, 3.5 years), and the remaining 30 patients were alive (n = 25, mean: 19.0 years) or had died (n = 5, mean: 9.5 years) of unrelated causes without evidence of disease. We conclude that extracranial ear and temporal bone meningiomas are rare tumors histologically similar to their intracranial counterparts. They behave as slow-growing neoplasms with a good overall prognosis (raw 5-y survival, 83%). Extent of surgical excision is probably the most important factor in determining outlook because recurrences develop in 28% of cases.

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.

Middle ear adenoma (MEA) is a rare tumor postulated to take origin from the lining epithelium of the middle ear cavity. The authors report on a case of MEA arising in a 53-year old woman suffering from a sensation of fullness in her left ear, otalgia, and light left-sided hearing loss. Histopathologically, the lesion was composed of cuboidal and polygonal cells displaying a trabecular, tubulo-glandular, and solid pattern of growth. Immunohistochemically, neoplastic cells diffusely stained with anti-vimentin antibodies and were focally positive for chromogranin A, neuron-specific enolase, lysozyme, and cytokeratins AE1/AE3. The majority of tumor cells showed weak and diffuse staining with both anti-PP and anti-ACTH antibodies and intense positivity with anti-glucagon and anti Leu-7 antibodies. Ultrastructural investigation revealed both mucinous-glandular and neuroendocrine differentiation. The authors suggest that the appropriate terminology would be adeno-carcinoid or amphicrine tumor of the middle ear rather than "adenoma," a term that does not reflect its dual nature.

We report three adenomatous middle-ear tumours, an adenoma, an adenocarcinoma and a semimalignant adenomatous tumour, with special attention to CT and MRI findings. In all cases we found small intratympanic masses in which the ossicles were embedded. All showed contrast enhancement and similar signal intensity as brain tissue on T1- and T2-weighted images. The biological nature of the tumours was not reflected by the imaging or operative findings.

Middle ear adenoma with neuroendocrine differentiation (carcinoid tumor) is a rare benign tumor of the tympanic cavity. Carcinoid tumor is generally derived from primitive stem cells in the gut wall but can be seen in other organs, including the lungs, mediastinum, thymus, liver, pancreas, bronchus, ovaries, prostate, and kidneys. This is unusual considering neuroendocrine cells have not been identified in normal or inflamed middle ear mucosa. Carcinoid tumor of the middle ear represents a class within the spectrum of adenomatous neoplasms. Immunohistochemical techniques have helped clarify that these tumors all share elements of neuroendocrine differentiation. Although histologic and biological similarities with middle ear adenoma exist, carcinoid tumor of the middle ear should be classified as a well-differen tiated neuroendocrine carcinoma because the carcinoid tumor has higher rate of recurrence and incidence of metastasis than middle ear adenoma. Surgical management is the recommended treatment and long-term observation is necessary. � Korean J Otorhinolaryngol-Head Neck Surg 2011;54:573-7 Key WordsZZMiddle ear ㆍCarcinoid tumor.

Middle ear adenomas are rare and difficult to diagnose. Recent histological studies suggest the presence of a distinct disease entity known as a middle ear adenoma. A 16-year-old boy with a middle ear tumor was treated by partial temporal bone resection, and the tumor was removed by a combined method using the middle cranial fossa and infratemporal fossa approaches. Histological and immunohistochemical studies on this tumor showed the findings of a benign adenoma. Left facial palsy was noted postoperatively, but it subsequently recovered almost completely.

Two cases of progressive hearing loss due to middle ear tumours are described. The histological characteristics numbered intraluminal mucin production and neuroendocrine features, as shown by argyrophilia and ultrastructural demonstration of dense core granules. These tumours have been known by many different names, reflecting the controversies relating to their presumed histogenesis and differentiation. The currently preferred designation is middle ear adenoma, and these two cases provide further evidence for dual lines of differentiation.