Middle ear adenoma with neuroendocrine differentiation (MEA-ND) in the pediatric population

Middle ear adenoma with neuroendocrine differentiation is an uncommon tumor of the tympanic cavity. The terminology of this entity has suffered due to a poor understanding of the differentiation of these neoplasms, and has included both “adenoma” as well as “carcinoid tumor.” Immunohistochemical techniques have helped clarify that these tumors all share elements of neuroendocrine differentiation. Occurrences of this tumor in the pediatric population are extremely rare. In the world literature, only three pediatric cases of middle ear adenoma with neuroendocrine differentiation have been described. We report the youngest case to date of middle ear adenoma with neuroendocrine differentiation, in a 13-year-old boy. We discuss the differential diagnosis, surgical management, and pathology of this entity.     

Middle ear adenoma diagnosed by recurrent facial paralysis

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: a case report

Middle ear adenoma (MEA) is a rare neoplasm with benign clinical behavior. The immunohistochemical and ultrastructural studies revealed the mixed, bidirectional mucinous and neuroendocrine character. These tumors have been known by many different names, reflecting the controversies relating to their histogenesis and differentiation. The term middle ear adenoma was proposed by Hyams and Michaels in 1976 and since this time over 100 cases were described in the literature. The most often symptoms of the MEA are the unilateral hearing loss and the tinnitus. MEA should be treated by surgery and very attentive postoperative follow-up. The prognosis of these tumors are good. The histological structure of middle ear adenoma, as well as symptomatology, treatment and clinical behavior are discussed on the basis of the literature. A case of histologically confirmed middle ear adenoma in a 34 year old women is presented.     

Carcinoid tumor of the middle ear: clinical features, recurrences, and metastases

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.     

Carcinoid tumor of the middle ear. Morphology, immunohistochemistry, clinical aspects and differential diagnosis

We describe the unusual case of a carcinoid tumour of the middle ear occurring in a woman of 50 years of age. The tumour exhibited both glandular and neuroendocrine differentiation. Immunohistochemical demonstration of chromogranin A is of particular diagnostic importance as evidence of neuroendocrine differentiation. The morphological spectrum of the middle ear tumours with neuroendocrine and/or glandular differentiation reported in the literature is discussed in detail. These tumours are arranged schematically according to the degree of glandular and of neuroendocrine differentiation, and are compared with the tumours of other regions. The data in the literature do not allow unequivocal assertions concerning the prognosis of carcinoid tumours of the middle ear to be made, and very diverse therapeutic measures have been employed in the reported cases. In our case there is no evidence of recurrence six months after local tumour excision.     

Neuro-endocrine adenoma of the middle ear: a case study

Neuroendocrine adenomas are rare tumors, which can appear in the middle ear. Approximately a hundred cases have been reported in the literature. We report the case of a 58-year-old man who consulted for an abnormal sensation of fullness in the right ear. The otoscopic examination showed a retrotympanic tumefaction. The CT scan and MRI of the middle ear demonstrated a well-defined tissue mass without any osteolysis. We performed surgical exeresis by transcanal procedure with a cartilage graft tympanoplasty. Microscopic examination and immunohistochemistry revealed an endocrine adenoma of the middle ear. Neuroendocrine adenomas can develop in a number of different sites. When they appear in the middle ear they usually produce hypoacousia. The otoscopic examination shows non-specific findings with only retrotympanic swelling. Surgical exeresis enables histologic and immunohistochemically analysis of the surgical specimen. The adenoma is composed of two cellular types: neuroendocrine (which closely resemble carcinoid tumors) and glandular. Regular clinical and radiologic follow-up is necessary since recurrence is possible. The formal diagnosis of neuroendocrine adenomas of the middle ear requires histologic and immunohistochemically confirmations since the clinical symptoms are non-specific. Surgical excision with removal of the ossicular chain is the treatment of choice in order to prevent recurrence.     

A case of syryngocystadenoma papilliferum (SCAP) involving middle ear, presenting with otomastoiditis and cerebral abscess

Syryngocystadenoma papilliferum (SCAP) is an uncommon cutaneous adnexal neoplasm of apocrine gland origin. Until now, fewer than 200 cases have been reported in international literature, of which 12 cases involved the external ear. No cases of SCAP involving the middle ear have been reported. We describe a case of SCAP of external and middle ear, presenting with otomastoiditis and cerebral abscess; this aggressive behavior, never reported for a benign adenoma of the external or middle ear, could lead to a differential diagnosis with cholesteatoma or malignancies.     

Middle ear adenoma with neuroendocrine differentiation

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.     

Carcinoid of the middle ear: a rare tumor with biphasic differentiation. Case report with special reference to immunohistochemistry and electron microscopy]

Progressive unilateral sensorineural deafness and tinnitus developed in a 59-year-old woman over a 1-year period. Clinical examination showed a tumor mass which almost completely filled the tympanic cavity, and grew around the auditory ossicular chain. Histological findings revealed the tumor to be a primary carcinoid of the middle ear. Neuro-endocrine differentiation was confirmed immunohistochemically by positivity for neuron-specific enolase, chromogranin, pancreatic polypeptide and synaptophysin. Using electron microscopy, neuroendocrine granules could be visualized. In addition, both light and electron microscopy revealed that cells had an epithelial differentiation with mucin granules while immunohistochemistry showed a positivity for cytokeratins. The detection of intermediary filaments (immunohistochemically with vimentin and under electron microscopy) was unique to this neoplasm and has to be considered in distinguishing the carcinoid tumor from the papillary adenoma of the middle ear. Tumor prognosis is excellent with radical extirpation from the middle ear. In the case presented, there has been no evidence for either recurrence or metastases 10 months after surgical resection.     

Amphicrine adenoma of the middle ear: three cases reports and a review of the literature

Amphicrine adenoma is an extremely rare tumor of the middle ear. We report three cases obtained over a twenty year period. THE AIM OF THIS STUDY: is to discuss our clinical cases and review the literature. MATERIALS AND METHODS: Three cases of middle ear amphicrine tumour were reviewed. RESULTS: During a 10 year period (1986-2006) three cases of middle ear amphicrine tumour were treated at our unit (2 male, 1 female). The presenting complaint was hearing loss in all 3 cases. Amphicrine adenoma is diagnosed by an immuno-histological examination of pathological specimens. Differential diagnosis can be difficult and one individual was initially treated as an adenocarcinoma by radiotherapy. This patient succumbed to cerebral mycosis two years post-radiotherapy. The other two patients have no evidence of recurrence eight and nine years post treatment. CONCLUSION: Historically, differential diagnosis between amphicrine adenoma and adenocarcinoma of the middle ear has been very difficult. Carcinoid tumour is considered to be a more agressive form of amphicrine adenoma. Tumour progression is through loco regional invasion. Complete excision by surgical management with long term follow up is the treatment of choice.     

Huge middle ear adenoma with delayed facial nerve paralysis

Middle ear adenoma is a rare disease that arises from the mucosa of the middle ear. Only a few cases of associated facial nerve paralysis have been reported. Facial nerve involvement is most likely related to nerve compression rather than tumor invasion of the nerve. We describe a case of a huge middle ear adenoma in a 63-year-old man. He presented with a 1-month history of right-sided otalgia, otorrhea, and facial palsy; he also had a 10-year history of right-sided hearing loss. A tympanomastoidectomy was performed. Intraoperatively, the tumor was found to fill the middle ear cavity as well as the entire diameter of the external auditory canal. The tumor had eroded the wall of the facial canal at the second genu, and it was tightly adherent to the epineurium. Focal inflammation around the tumor was observed at the exposed facial nerve. The tumor was removed and the facial nerve was decompressed. Immediately after surgery, the patient's aural symptoms resolved. The final pathology evaluation established the diagnosis of a middle ear adenoma. At the 3-year follow-up, the ear cavity was completely healed and facial nerve function was improved.     

Adenoma of the middle ear

Benign adenoma of the middle ear emerged as a distinct entity following a review of 20 cases as reported from the Armed Forces Institute of Pathology in 1976. We report 4 additional cases and note that the pathologic diagnosis is not so clear cut. Benign primary adenoma may be confused with adenocarcinoma, ceruminoma, and glomus tumor. The otologic surgeon must take a hard look at the clinical signs and symptoms to determine if they are compatible with the diagnosis of primary middle ear adenoma. While middle ear adenoma has recently been implicated as a cause of facial paralysis (including a case herein), bone destruction should never be found in concert with this neoplasm.     

Carcinoid tumor of the middle ear: report of a case

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 from the middle ear adenoma. However, the balance of opinion is currently on 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 wall-down procedure. The relevant literature is also herewith reviewed.     

Karzinoid des Mittelohrs Weitere Therapie nach primärer Tumorexstirpation?

The carcinoid tumor of the middle ear is a very rare neoplasm which in general is regarded as benign but may be mistaken for an adenocarcinoma because of its histological heterogeneity. Typical, however, is its neuroendocrine and mucinous differentiation so that an unequivocal diagnosis is possible by means of immunohistochemistry and electron microscopy. As the tumor is very rare, there is no statistical evidence as to whether further treatment is necessary after primary exstirpation of the tumor. Therefore, a review of the literature was performed. We report about a 28-year-old male patient with a carcinoid tumor of the left tympanic cavity. Without any further treatment there has been no evidence for either recurrence or metastases 32 months after surgical resection. As treatment of choice we recommend conservative surgery with complete removal of the tumor and a clinical follow-up on a regular basis.     

Neuroendokrine Adenome im Mittelohr

Background

Neuroendocrine adenomas of the middle ear are rare, low-grade neoplasms with potential for recurrence and metastasis. The nonspecific symptoms and preliminary clinical and radiological findings are misleading and often fail to provide the right diagnosis.

Patients and methods

We analyzed the findings of 3 adult patients, who were treated between 2001 and June /2012 at the Luzerner Kantonsspital in Switzerland.

Results

The 3 patients reported on hearing loss, ear pressure and/or tinnitus. Otoscopy showed a thickened, intact tympanic membrane with a whitish–grayish prolapsing mass. All the patients had conductive hearing loss. Computer tomography showed an unspecified well-circumscribed soft-tissue mass. Definitive histology with immunostaining after radical tumor removal led to the proper diagnosis.

Conclusions

The typical constellation of nonspecific clinical and radiological findings leads to the right diagnosis of neuroendocrine adenoma of the middle ear. After reviewing the literature, we illustrate the differential diagnosis as well as the relevant diagnostic and therapeutic procedures, and remind ENT physicians about this rare disease entity.     

Carcinoid tumor of the middle ear

Although carcinoid tumors are labeled as neuroendocrine tumors they can also originate in tissue lacking neuroendocrine cells, such as that in the middle ear. Symptoms of a carcinoid tumor in the middle ear are common ear symptoms such as fullness, pain and hearing loss. Carcinoid tumors have also been considered to be slow-growing. Both these aspects can easily lead to a relatively late diagnosis of carcinoid tumor of the middle ear. The diagnosis is made histologically, and the tumor is primarily treated surgically. In the follow-up of patients, octreotide scanning has proved to be a sensitive method in cases of both recurrence and metastasis. Our patient was a 34-year-old, otherwise healthy female with left-sided acute otitis media and facial palsy in her left ear. She had also suffered from the same symptoms 4 years earlier. She was treated with an operation, and the histologic diagnosis was a carcinoid tumor. In the follow-up of the patient we used octreotide scanning.     

Carcinoid tumor of the middle ear

Although carcinoid tumors are labeled as neuroendocrine tumors they can also originate in tissue lacking neuroendocrine cells, such as that in the middle ear. Symptoms of a carcinoid tumor in the middle ear are common ear symptoms such as fullness, pain and hearing loss. Carcinoid tumors have also been considered to be slow-growing. Both these aspects can easily lead to a relatively late diagnosis of carcinoid tumor of the middle ear. The diagnosis is made histologically, and the tumor is primarily treated surgically. In the follow-up of patients, octreotide scanning has proved to be a sensitive method in cases of both recurrence and metastasis. Our patient was a 34-year-old, otherwise healthy female with left-sided acute otitis media and facial palsy in her left ear. She had also suffered from the same symptoms 4 years earlier. She was treated with an operation, and the histologic diagnosis was a carcinoid tumor. In the follow-up of the patient we used octreotide scanning.     

Tumors arising from the glandular structures of the external auditory canal

Neoplasms in the external auditory canal (EAC) of ceruminous gland origin have been generally classified under the title of ceruminoma, which is inaccurate and misleading. There have emerged four distinct types of ceruminous gland tumors of the EAC. They are 1. ceruminous adenoma, 2. adenoid-cystic carcinoma, 3. ceruminous adenocarcinoma, and 4. pleomorphic adenoma (mixed tumor). The natural course and clinical approach to these tumors can be determined by accurate histopathologic evaluation. This paper presents 10 cases of tumors of glandular origin in the EAC, 4 cases being ceruminous adenomas, 3 cases being adenoid-cystic carcinomas, 2 cases being ceruminous adenocarcinoma, and 1 case of pleomorphic adenoma (mixed tumor). In reviewing these cases as well as those in the literature, a number of recommendations are suggested: 1. Identifying a tumor of the glandular structures of the EAC solely as a ceruminoma is no longer acceptable without accompanying histologic specificity. 2. Early wide excisional biopsy is imperative for diagnosis. 3. The signs and symptoms of the tumor do not always correlate with the histopathologic diagnosis and subsequent clinical behavior of these tumors. 4. Ceruminous adenoma and pleomorphic adenoma are benign tumors and are best treated only by wide local excision. 5. Adenoid-cystic carcinoma and ceruminous gland adenocarcinoma are pernicious, malignant tumors which are best treated, in general, by an initial aggressive wide en bloc surgical resection or, if there is extension to the middle ear and temporal bone, by resection of the temporal bone and contiguous structures. 6. Postoperative irradiation has an essential role in managing these malignant tumors. 7. Five year survival rates for the malignant tumors do not reflect the biological behavior pattern of "late" local and distant recurrence and metastasis.     

Aggressive papillary tumor of the middle ear: a true entity or an endolymphatic sac neoplasm?

Aggressive papillary tumors of the middle ear are rare, and their true origin is not clear. We describe the clinical, radiologic, genetic, and histopathologic features of a papillary epithelial tumor filling the middle ear of a 68-year-old woman. Imaging revealed no evidence of petrous temporal bone apex involvement, nor did genetic studies demonstrate the von Hippel-Lindau mutation. A literature search revealed 24 previously reported cases of such a papillary epithelial tumor of the middle ear. All except 2 cases demonstrated apical petrous temporal bone invasion on imaging, and it has been suggested that they arose from a primary endolymphatic sac tumor, which has a similar papillary epithelial histology. Substantial numbers of cases of papillary epithelial tumors involving the middle ear are reported to be associated with von Hippel-Lindau disease, as are known cases of endolymphatic sac tumor. This is the third reported case of papillary epithelial tumor of the middle ear that does not show apical petrous temporal bone invasion on imaging, suggesting that such neoplasms do not always arise from a primary in the endolymphatic sac.     

Adenomatous tumors of the middle ear and mastoid

Adenomatous tumors of the middle ear and mastoid have been called adenoma or adenocarcinoma. The clinical and pathologic distinction between the two has been difficult. The resultant pathologic ambiguity makes it difficult to decide whether conservative or radical management is appropriate. The Otologic Medical Group's (OMG) experience with glandular tumors of the middle ear and mastoid over the past 27 years was reviewed. Thirteen cases were found and analyzed with respect to signs and symptoms at presentation. Extensive histopathologic review with light and electron microscopy was performed on tumor specimens. Two distinctive histopathologic and clinical patterns were identified. The mixed type of tumor was always confined to the middle ear and mastoid, was commonly misdiagnosed as chronic otitis media, and rarely demonstrated otic capsule or facial nerve involvement. The papillary pattern always had extension to the petrous apex and frequently involved the middle and/or posterior cranial fossa. Papillary tumors were more frequent in females and usually involved the facial nerve. On the basis of the findings in this review as well as information from the literature, we have come to the following conclusions: 1. The correct general pathologic heading be Adenomatous Tumors of the Middle ear and Mastoid with each tumor then being subclassified into Mixed or Papillary tumor and adenocarcinoma when warranted by histology. 2. There is a high rate of local recurrence. 3. Long-term follow-up (at least 10 years) for all adenomatous tumors is necessary. 4. Primary surgical treatment is required.