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This morphological and immunohistochemical study demonstrates that tumors currently known as "middle ear adenomas" are truly well-differentiated epithelial neuroendocrine tumors (NETs) composed of cells comparable to normal intestinal L cells, and therefore, these tumors resemble hindgut NETs. These tumors show consistent expression of glucagon, pancreatic polypeptide, PYY, and the transcription factor SATB2, as well as generic neuroendocrine markers and keratins. The same L cell markers are expressed by cells within the normal middle ear epithelium. These markers define a valuable immunohistochemical profile that can be used for differential diagnosis of middle ear neoplasms, particularly in distinguishing epithelial NETs from paragangliomas. The discovery of neuroendocrine cells expressing the same markers in non-neoplastic middle ear mucosa opens new areas of investigation into the physiology of the normal middle ear and the pathophysiology of middle ear disorders.

Despite its rarity, the neuroendocrine adenoma of the middle ear (NEAME) is a differential diagnosis of the middle ear disease. We suspect that the NEAME is underdiagnosed and more cases can be detected through education and personal experience. Treatment is R0 resection, and long follow‐up is recommended.

Endolymphatic sac tumour (ELST) is a rare low grade malignant epithelial tumour of the petrous temporal bone, thought to arise from papillary epithelium of the endolymphatic sac. They may occur sporadically or in association with Von-Hippel Lindau disease. ELST is extremely rare neoplasm with benign histopathological appearance and clinically destructive behaviour. Because of the rarity of this tumour, it can easily be confused with other tumours such as paraganglioma, middle ear adenoma, metastatic carcinomas or choroid plexus papilloma. We report here a rare case of ELST with review of literature and discuss the differentiating features of ELST from its mimickers, showing a papillary configuration.

Neuroendocrine adenoma of the middle ear (NAME) represents a rare tumour consisting of an adenoma with mixed neuroendocrine differentiation. A 40-year-old woman was referred to our attention to further investigate the occurrence of a pathological tissue located in the mastoid process of the left temporal bone depicted by head CT and MRI scans. Histopathological examination revealed an epithelial neoplasm with neuroendocrine differentiation features, consistent with the diagnosis of NAME. In order to obtain an accurate differential diagnosis and confirmation of this rare disease, 111In-Octreoscan single photon emission computed tomography (SPECT)/CT and 68Ga-DOTANOC positron emission tomography (PET)/CT were performed, both showing overexpression of somatostatin receptors and thus corroborating the histopathological findings.

BACKGROUND Paragangliomas (PGLs) are rare neoplasms that may be associated with hereditary PGL syndromes and variable risk of metastasis. Middle ear adenomas are extremely rare tumors with no known hereditary predisposition and extremely low risk of metastasis. Although often easily differentiated, they may share clinical and pathological features that misdirect and confuse the diagnosis. OBSERVATIONS The authors discussed a 35-year-old woman with left-sided hearing loss and bleeding from the external ear canal who presented to an outside hospital. She underwent resection of a middle ear and mastoid mass, initially diagnosed as a middle ear adenoma with neuroendocrine features, with later mastoidectomy and ligation of the sigmoid sinus with microsurgical excision of persistent tumor in the jugular foramen and temporal bone. Histopathologically, her tumor was vascular, composed of benign-appearing epithelioid cells with “salt and pepper” neuroendocrine chromatin arranged in vague nests. Lesional cells were GATA3-immunopositive, glucagon-negative, and succinate dehydrogenase-immunonegative, consistent with PGL rather than middle ear adenoma, and required further workup for hereditary PGL syndromes. LESSONS This case demonstrates potential challenges in differentiating a PGL from a middle ear adenoma. The authors offer clinical, histopathological, and imaging principles to aid in diagnosis and workup.

Background: Syringocystadenoma papilliferum is a rare adnexal tumor of controversial origin that often occurs as a solitary tumor derived from apocrine and eccrine glands. It is more common in children and adolescents and affects the head and neck regions,although occurrences on other anatom-ical locations have been described. Routine H & E-stained sections revealed cystic epidermal invaginations with papillary projections. The superficial portions of the cyst were lined by stratified keratinizing epithelium, whereas the deeper papillated portion exhibited a double layer of basal-like cells and luminal eosinophilic columnar cells with focal decapitation secretions.Patient and Method: A female patient, aged 29 years, presented to us in the outpatient clinic suffering from left earache and hearing loss since the past 3 months, with gradual onset and a slowly progressive course. On examination, we found a left aural polyp, which was firm and grayish in color, but extremely tender on touch. Biopsy for histopathological examination was consistent with a diagnosis of syringocysta-denoma papilliferum. The patient received regular follow-up and is doing well with regard to her earache with marked hearing improvement. To conclude, solitary lesions seen in unusual locations generate multiple differential diagnoses and should be confirmed by histopathology in order to exclude other pathologies and define the appropriate therapeutic approach.Conclusion: Solitary lesions seen in unusual locations generate multiple differential diagnoses and they must be confirmed by histopathology

Summary Middle ear adenomas with neuroendocrine features (ANEF) are rare, with an estimated 150 reported cases. They usually pursue an indolent clinical course. Four reported cases of middle ear ANEF with distant metastases were treated with surgery, external beam radiation therapy (EBRT) and chemotherapy. To date, no successful systemic treatment for malignant behaviour of this rare tumour has been reported. Long-acting somatostatin analogues (SSAs) and peptide receptor radionuclide therapy (PRRT) have been used in well-differentiated metastatic neuroendocrine tumours (NETs), but their use has never been described in cases of metastatic middle ear ANEF. We report two patients with grade 1 middle ear ANEF treated with surgery and EBRT. They had stable disease for several years, until clinical symptoms appeared and extensive metastases were detected on 68Ga-DOTA0-Tyr3-octreotate (DOTATATE) PET/CT. Treatment with long-acting SSA was started, with stable disease for 1 year. Afterwards, despite undergoing local treatments, both patients presented progressive disease. Due to high-uptake metastases at 68Ga-DOTATATE PET/CT, both cases underwent four cycles of PRRT with 177Lu-DOTATATE, which secured disease control and improvement of quality of life in both. Similar to other well-differentiated NETs, SSA and PRRT could constitute efficacious therapeutic options in metastatic middle ear ANEF. Its neuroendocrine differentiation, potential to metastasize and somatostatin receptor type 2 expression prompt consideration and management of this disease as a neuroendocrine neoplasm. Learning points Our cases oppose the 2017 WHO classification of middle ear adenoma with neuroendocrine features as a benign disease. This entity warrants long-term follow-up, as local recurrence or persistence of disease is reported in up to 18% of surgically treated patients. PET/CT scan with 68Ga-labelled somatostatin analogues (SSA) can be used for staging of metastatic middle ear adenoma with neuroendocrine features. Unlabelled SSA and peptide receptor radionuclide therapy (PRRT) with radiolabelled SSA can be the first systemic therapeutic options for patients with advanced middle ear adenoma with neuroendocrine features.

International audience; Middle ear adenomatous neuroendocrine tumours are extremely rare causes of middle ear masses. Differential diagnosis is of primary importance to decide on the correct management since they require surgery whereas other lesions can be actively watched.CT and MRI show a non‐hypervascular lesion, differentiating it from paraganglioma. It is a mesotympanic lesion that usually encases the ossicles without bone erosion and it can invade the retrotympanum and Eustachian tube.Radical surgery is the treatment of choice. Since middle ear adenomatous neuroendocrine tumours adhere to adjacent structures and lack a tumour capsule, gross total resection is challenging.Late recurrences may be observed and complicated by the development of middle ear cholesteatoma, emphasising the importance of a long follow‐up.Recurrence can be associated with neural and intracranial invasion, lymph node or distant metastasis, highlighting the malignant potential of middle ear adenomatous neuroendocrine tumours.

Most reported cases of middle ear adenoma (MEA) have focused on histopathology because MEA is usually diagnosed postoperatively, which is considered as a major setback. We focused on the surgical aspect of the disease to facilitate a preoperative diagnosis, resulting in prompt and proper treatment, without requiring a second stage of surgical treatment. In this report, we present the differential diagnoses in a 40-year-old man with MEA requiring surgical treatment. Preoperatively, the patient was suspected to have an MEA. An analysis of the surgical procedures in similar misdiagnosed tumors has enabled us to assess surgical procedures in cases wherein the preoperative diagnosis does not coincide with the postoperative histopathological results.

A 52-year-old man presented to our emergency department with an acute onset of right-sided facial nerve (FN) palsy of House-Brackmann grade V. Electroneurography (ENoG) was conducted with no response at the right FN, as compared with the left FN (0%). We performed a biopsy of the right middle ear mass and histological studies showed the tumor to be neuroendocrine tumors (NET) of the middle ear. We resected the tumor with canal wall down mastoidectomy and reconstructed the posterior meatal wall with soft tissue. Three months after surgery, the FN paralysis had improved with House-Brackmann grade II. We reviewed cases of NET with FN palsy, and nine patients, including our case, have been reported. Our case is the first report of ENoG for the description of FN palsy due to NET. Although the ENoG value was 0%, it was remarkably improved by surgery. The other cases of NET patients with FN palsy also recovered FN function after surgery. These results suggest that it is recommended to perform the total resection of the tumor to improve the FN function.

Here, we present a rare case of middle ear adenoma in a 58-year-old female, which uniquely presented as an unstable open cavity after an endaural atticoantrotomy. Immunohistochemistry confirmed that the tumor produced endocrine and exocrine secretions. The tumor was radically surgically removed.

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.

Introduction: Inspite of modern radiological work up, surgeons can still be surprised by intraoperative findings or by pathologists report. Materials and methods: We present the case history of a 51 year old male who came to our institute with complaints of unilateral hearing loss, involuntary facial movements and retroauricular pain for a period of 1 year. He underwent Fisch approach with short segment mobilization of facial nerve. Results: Though pre-operative CT and MRI findings were suggestive of a petroclival mass he had middle ear adenoma. Key Words: Middle ear, Middle Ear Adenoma, Middle Ear benign tumor, Adenoma

The ear comprises several structures that are the source of pathology including the skin, bone, cerumen glands, and specialized cochlear tissues. Its location and development make it a unique source of rare tumors, developmental abnormalities, and hereditary syndromes. This chapter will cover the limited but rare pathologic conditions and tumors of the external, middle, and inner ear.

Tantamount to the management of temporal bone neoplasms is the ability to visualize the pathology and its relationship with the numerous critical structures housed therein. Transcanal endoscopic ear surgery provides the surgeon with an unparalleled view of the entire middle ear. This article presents the latest information on the usefulness of transcanal endoscopic ear surgery in the management of middle ear and temporal bone neoplasms.

Introduction: Carcinoid tumor of the middle ear (ME) is exceedingly rare and thus its diagnosis is frequently delayed. It is also hard to differentiate between middle ear carcinoid from the middle ear adenoma. Defi nitive diagnosis is made by identifying neurosecretory tumor cells using immunohistochemistry and electron microscopy.

Middle ear adenoma with neuroendocrine differentiation (MEA-ND) is also called as neuroendocrine adenoma. Neuroendocrine tumors are rarely seen in the head and neck region and are even more rare in the middle ear. Clinical and radiological findings are non-specific and seldom suggest this diagnosis. Nomenclature and behavior of this tumor has been historically controversial. Both epithelial as well as neuroendocrine origin have been suggested. They comprise

Adenomatous tumors are an uncommon cause of a middle ear mass. Clinical findings may be nonspecific, leading to difficulties in differentiation from other middle ear tumors. Controversy also exists whether to classify middle ear adenoma and carcinoid as separate neoplasms, or alternatively within a spectrum of the same pathologic entity. Most adenomatous middle ear tumors are indolent in behavior, with a benign histologic appearance and slowly progressive growth. The mainstay of treatment is complete surgical resection, which affords the greatest likelihood of cure.

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.

Middle ear adenoma with neuroendocrine differentiation (MEA-ND), also known as carcinoid tumor of the middle ear, is an extremely rare neoplasm, especially when located within the tympanic cavity. To the best of our knowledge, this represents the first pediatric case of a MEA-ND described in Europe. Excluding the present case, only three other pediatric cases of this disease have been published globally. We report on a 15-year-old female patient who presented with conductive hearing loss of the right ear. A tympanogram was flat. Ear microscopy revealed bulging of the right posterior superior tympanic quadrant. Computed tomography depicted an opacification of the middle ear and mastoid air cells without evidence of bone erosion. Paracentesis was performed assuming a serous otitis media. Since there was no liquid found the patient underwent an atticoantrotomy and mastoidectomy as therapy for cholesteatoma. Histologic examination revealed a highly differentiated neuroendocrine tumor. Immunohistochemical evaluation showed strong positivity for synaptophysin. On the basis of these results, the patient underwent a middle ear revision surgery. After 15 months, magnetic resonance imaging and octreotide scans suggested disease recurrence, prompting a second surgical revision. The histologic examination confirmed a recurrence of only 1 mm, but postoperative imaging still showed pathological diffuse enhancement. Eight years after the initial diagnosis there has been no evidence of recurrence or metastasis. In our opinion a pathological radiological result is insufficient to identify recurrent or residual disease within the tympanic cavity.

Neuroendocrine tumours occur throughout the body but are rare in the head and neck region and particularly rare in the middle ear. Clinical findings are often nonspecific and therefore pose a diagnostic challenge. Furthermore, the nomenclature of neuroendocrine tumours of the middle ear is historically controversial. Herein a case is presented of a middle ear adenoma in a 33-year-old patient who presented with otalgia, hearing loss, and facial nerve palsy. A brief discussion is included regarding the histopathological features of middle ear adenomas and seeks to clarify the correct nomenclature for these tumours.