Middle Ear "Adenoma": a Neuroendocrine Tumor with Predominant L Cell Differentiation.

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.

New proposal to revise the classification for squamous cell carcinoma of the external auditory canal and middle ear.

BACKGROUND: The prognosis of patients with advanced squamous cell carcinoma of the external auditory canal and middle ear has been improved by advances in skull base surgery and multidrug chemoradiotherapy during the last two decades. METHODS: Ninety-five patients with squamous cell carcinoma of the external auditory canal and middle ear who were treated between 1998 and 2017 were enrolled. The number of patients with tumour stages T1, T2, T3 and T4 was 15, 22, 24 and 34, respectively. Oncological outcomes and prognostic factors were retrospectively investigated. RESULTS: Among patients with T4 disease, invasion of the brain (p = 0.024), carotid artery (p = 0.049) and/or jugular vein (p = 0.040) were significant predictors of poor prognosis. The five-year overall survival rate of patients with at least one of these factors (T4b) was significantly lower than that of patients without these factors (T4a) (25.5 vs 65.5 per cent, p = 0.049). CONCLUSION: It is proposed that stage T4 be subclassified into T4a and T4b according to the prognostic factors.

Update From the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Tumours of the Ear.

The 2017 fourth edition of the World Health Organization Classification of Tumours, specifically as it relates to the ear (Chap. 9), has several changes. Importantly, the number of entities has been significantly reduced by omitting tumors or lesions if they do not occur exclusively or predominantly at this site or if they are discussed in detail elsewhere in the book. These entities include: embryonal rhabdomyosarcoma, osteoma, exostosis, angiolymphoid hyperplasia with eosinophilia, Schneiderian papilloma, inverted papilloma, lipoma of the internal auditory canal, hemangioma, hematolymphoid tumors, and secondary tumors. Paraganglioma was included in the neck chapter. New entries include otosclerosis and cholesteatoma, while refinements to nomenclature, classification and criteria were incorporated into the ceruminous gland tumors and epithelial tumors of the middle and inner ear. Specifically, the middle and inner ear were combined, as practical limitations of origin and imaging make a definitive separation artificial. The classification reflects the state of current understanding for these uncommon entities, with this update only highlighting selected entities that were the most significantly changed.

Carcinoma espinocelular del oído externo con extensión parotídea: a propósito de un caso / Squamous cell carcinoma of the external ear with parotid extension: case report

El pabellón auricular y el conducto auditivo externo constituyen una región anatómica que puede ser asiento de múltiples patologías, entre ellas procesos inflamatorios, infecciosos y neoplásicos, tanto benignos como malignos. Con respecto a los tumores, los diversos tipos suelen presentarse con síntomas y signos similares y en general es difícil inferir la variedad histológica del tumor a través del examen físico, por lo cual es necesario el estudio histopatológico para determinar el diagnóstico. La mayoría de los tumores del oído externo son carcinomas; entre ellos se destacan el carcinoma basocelular, el más frecuente, y el carcinoma espinocelular. Menos frecuentemente se encuentran otros tipos de tumores como los melanomas, adenocarcinomas, carcinomas de glándulas ceruminosas, carcinomas mucoepidermoides, sarcomas, procesos linfoproliferativos, etc. Suelen ocurrir en la edad media y avanzada (50-70 años) y con mayor periodicidad en el sexo masculino. En el presente trabajo se describe un caso clínico de carcinoma espinocelular del oído externo, tratado exitosamente mediante cirugía y radioterapia, así como también se describen las características clínicas de esta enfermedad, con especial atención al compromiso del oído externo por ella.

The pinna and the ear canal are an anatomical region that can be affected by many diseases, including inflammatory, infectious and benign and malignant neoplastic processes. With regard to tumors, various types usually present with similar symptoms and usually is very difficult to know the histological type through physical examination, so histopathological examination is necessary in order to determine the diagnosis. Most tumors are carcinomas; they can be basal cell carcinoma (more frequently), or squamous carcinoma. Less frequently are other types of tumors such as melanomas, adenocarcinomas, ceruminous glands carcinomas, mucoepidermoid carcinomas, sarcomas, lymphoproliferative disorders, etc. They usually present in middle and advanced age people (50-70 years) and are more frequently in men. In this article we present a case of squamous cell carcinoma of the external ear with extention to parotid gland, successfully treated with surgery and radiotherapy, as well as we describe the clinical characteristics of this disease, with special attention to the compromise of the external ear. (AU)

Adenomatous tumors of the middle ear.

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.

Vertebral artery involvement by tympanojugular paragangliomas: management and outcomes with a proposed addition to the fisch classification.

OBJECTIVES: To present the management and outcomes of cases of tympanojugular paraganglioma (TJP) with vertebral artery (VA) involvement, and to propose the addition of the new 'V' category to the Fisch classification. MATERIALS AND METHODS: Retrospective analysis of data from a quaternary neuro-otologic and skull base referral center. We studied 230 TJP patients to identify the cases with VA involvement and present their management, surgical findings and outcome. RESULTS: Out of 230 patients, 8 patients were found to have VA involvement by TJP. The extradural artery was involved in 1 patient and the intradural VA was involved in 6 patients. The intradural and extradural VA were simultaneously involved in 1 patient. Seven of the 8 patients underwent surgery. In 2 patients, preoperative occlusion was performed (1 with balloon, 1 with coils). In 5 of these 7 patients, the tumor was successfully separated from the VA by microdissection with or without endovascular intervention. In another patient, the tumor together with the involved VA was removed. Of 7 patients who had surgery, 1 patient did not undergo endovascular intervention and had subtotal tumor removal from the VA. There were no serious complications in removing the tumor from around the VA. CONCLUSION: Although uncommon, the intradural or extradural VA can be involved by TJP. To avoid vascular accidents, the VA should be thoroughly evaluated at the planning stage. We advocate that the addition of a 'V' category would help to identify patients who need individualized VA management as part of a complete surgical evaluation.

Intralabyrinthine schwannomas: imaging diagnosis and classification.

BACKGROUND AND PURPOSE: ILS is a rare lesion that has a different management from the more common "acoustic" schwannoma. To date, only 137 cases have been reported. We present a classification scheme based on labyrinthine anatomy to describe and localize these lesions. Treatment and prognosis hinge on the appropriate localization of these tumors; thus, a concise terminology that can be used by both the otolaryngologist and radiology communities is desirable. MATERIALS AND METHODS: After approval of the institutional review board, a retrospective study of all patients with the diagnosis of ILS imaged between 1996 and 2010 was performed. Clinical and imaging data were collected. Patients were imaged with thin-section high-resolution T2 and contrast-enhanced MR imaging. RESULTS: There were 45 patients with a diagnosis of ILS. Forty-three had complete histories. There were 18 male and 25 female patients with an age range of 21-78 years with a mean age of 53 years. The most common presenting symptom was progressive sensorineural hearing loss. Lesions were characterized on the basis of their location. Intracochlear was most common (14/45) followed by transmodiolar (13/45), intravestibular (7/45), vestibulocochlear (5/45), transmacular (4/45), and transotic (2/45). Sixteen patients underwent surgical resection. The remaining patients were followed clinically and by serial MR imaging. CONCLUSIONS: ILS is an uncommon but under-reported tumor. We characterized the MR imaging appearance of these tumors by using high-resolution techniques. In addition, an anatomically based classification system is presented that will help the radiologist accurately describe ILS within the inner ear and help the surgeon determine which tumors are potential surgical candidates.

The role of facial palsy in staging squamous cell carcinoma of the temporal bone and external auditory canal: a comparative survival analysis.

OBJECTIVES: The role for facial palsy in the Pittsburgh staging system for squamous cell carcinoma (SCC) of the external auditory canal (EAC) is unclear. The objective of this study was to conduct a systematic review of published studies to determine the impact of facial palsy on survival outcomes. DATA SOURCES: A search of MEDLINE, EMBASE, Cochrane Central Register of Clinical Trials, Cochrane, clinicaltrials.gov, and the National Guideline Clearinghouse databases was supplemented by hand searching. STUDY SELECTION: Articles selected for final analysis had individual subject data on staging and/or facial nerve function, outcome, and follow-up period. DATA EXTRACTION: Data extracted included demographics, type and stage of cancer, survival, and facial nerve status. DATA SYNTHESIS: Of 3,046 citations identified by a systematic literature search, 21 case series including 348 subjects with SCC of the EAC met criteria for analysis. The overall and disease-specific survival for subjects with facial palsy were significantly worse than subjects without facial palsy, regardless of stage (p = 0.006 and p = 0.002, respectively). The overall survival outcome for subjects with facial palsy was significantly worse than subjects with stage PITT-2000 T3-designated cancer (p = 0.027) and demonstrated no statistically significant difference from stage PITT-2000 T4-designated cancer (p = 0.897). CONCLUSION: This pooled-data survival analysis for SCC of the EAC demonstrates that facial nerve involvement is associated with a poor outcome and that the survival outcomes for subjects with facial palsy more closely parallel the survival curves of advanced stage T4 disease. Disease with facial palsy should be classified as stage T4, in accordance with the PITT-2000 system.

Low-grade adenocarcinoma of endolymphatic sac origin.

We present a case of papillary cystic low-grade adenocarcinoma of endolymphatic sac origin. These tumors are very rare and only a few cases have been reported in literature. They have a protracted clinical course causing progressive invasion and bony destruction. Radiologically, they appear as a heterogenous lobulated vascular mass, invading bone and compressing surrounding structures. Histologically, these tumors are composed of uniform population of cells, resembling normal endolymphatic sac epithelium. This causes considerable diagnostic difficulty. A strong index of suspicion along with clinical and radiological correlation is essential to arrive at a correct diagnosis. In some bilateral cases, association with von Hippel-Lindau disease has been noted. Radical mastoidectomy and temporal bone resection, which may sometimes necessitate sacrifice of cranial nerves, is the treatment of choice.

Can E-selectin be a reliable marker of inflammation in lumbar disc disease?

The cause of sciatica and low back pain associating with lumbar disc herniation has not been clearly identified until now. Inflammation has been shown to occur via immunohistochemical and biochemical methods in herniated disc tissues. The important prognostic role of E-selectin has recently been substantiated by other studies in early rheumatoid arthritis (RA) and juvenile rheumatoid arthritis (JRA). The important role of adhesion molecules in the initiation and progression of the inflammatory response is well known for infectious diseases and autoimmune disorders. In our study, we aimed to show the role of E-selectin as an inflammatory marker and the correlation of inflammation with straight-leg raise (SLR) test findings and subtype of disc herniation. We found that the cases with positive SLR test had higher rates of immunostaining with E-selectin. This led us to think that E-selectin might play an important role in the activity status of the disease, meaning patients with more limited movement capacity might benefit from E-selectin antagonist therapy. Among the many studies performed to identify the relationship between the inflammation markers and activity of lumbar disc herniation, this is the first investigation held with E-selectin.

Posterior petrous meningiomas: 82 cases.

OBJECT: The aim of this study was to discuss posterior petrous meningiomas--their classification, clinical manifestations, surgical treatments, and patient outcomes. METHODS: A retrospective analysis was performed in 82 patients with posterior petrous meningiomas for microsurgery. According to the anatomical relationship with the posterior surface of the petrous bone and with special reference to the internal auditory canal (IAC), posterior petrous meningiomas were classified into three types: Type I, located laterally to the IAC (28 cases); Type II, located medially to the IAC, which might extend to the cavernous sinus and clivus (32 cases); and Type III, extensively attached to the posterior surface of the petrous bone, which might envelop the seventh and eighth cranial nerves (22 cases). Sixty-eight (83%) of 82 cases involved total resection. The rate of anatomical preservation of facial nerve was 97.5%, whereas the functional preservation rate was 81%. The rate of hearing preservation was 67%. All Type I tumors were completely resected, and the rate of anatomical preservation of facial nerve was 100% and functional preservation was 93%. Regarding Type II lesions, 75% of 32 cases involved total resection; the rate of anatomical preservation of facial nerve was 97% and functional preservation was 75%. For Type III lesions, 73% of 22 cases were totally resected. The rate of anatomical preservation of facial nerve in patients with this tumor type was 95%, whereas functional preservation was 73%. CONCLUSIONS: Clinical manifestations and surgical prognoses are different among the various types of posterior petrous meningiomas. It is more difficult for Types II and III tumors to be resected radically than Type I lesions, and postoperative functional outcomes are significantly worse accordingly. The primary principles in dealing with this disease entity include preservation of vital vascular and central nervous system structures and total resection of the tumor as much as possible.

The characteristic histopathologic features of nevi on and around the ear.

BACKGROUND: Nevi on certain areas of the body such as the acral, genital, and flexural regions may exhibit uncommon but characteristic histopathologic features. The purpose of this study was to characterize the distinctive features of nevi with a junctional component located on and around the ear. MATERIALS AND METHODS: A total of 101 compound and junctional nevi of the ear received at the Yale Dermatopathology Laboratory during a 10-year period were examined in this study. The most characteristic feature of the majority of these nevi was irregularity of nesting pattern, with nests, which varied in size and shape and which were sometimes located between rete ridges. RESULTS: Forty-two (42%) of nevi on and around the ear showed poor circumscription, lateral extension of the junctional component beyond the dermal component, and elongation of rete ridges with bridging between them. A subset of these nevi (26 cases) showed uniformly large melanocytes with large vesicular nuclei without prominent nucleoli, and abundant pale, finely granular cytoplasm. These lesions did not show a tendency to recur. CONCLUSIONS: This study supports the existence of a subset of nevi on or near the ear that, like certain nevi located on other special sites, exhibit unusual but characteristic features, which may be misinterpreted as atypical or malignant.

Intralabyrinthine schwannomas: diagnosis, management, and a new classification system.

OBJECTIVE: To outline the diagnosis and management of intralabyrinthine schwannomas and to propose a new classification system to further define them. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center at a university hospital. PATIENTS: Twenty-eight patients seen at the institution with intralabyrinthine schwannomas between 1996 and 2002 were included in the study. INTERVENTIONS: Diagnosis was made with magnetic resonance imaging in all but one case. Our ability to detect these tumors has been greatly enhanced by the introduction of high-resolution T2-weighted magnetic resonance imaging. Treatment options were observation with serial magnetic resonance imaging versus complete surgical removal via a translabyrinthine or transotic approach. Surgery was indicated for dizziness caused by the tumor or extralabyrinthine growth. MAIN OUTCOME MEASURES: Clinical features, audiology, radiology, and management outcomes were evaluated. RESULTS: Eight patients have undergone successful surgery with removal of their tumors and resolution of symptoms. One patient chose to have stereotactic radiotherapy. Of the 20 patients who were managed with observation and serial magnetic resonance imaging, only 1 has shown significant growth requiring surgical removal. CONCLUSION: Intralabyrinthine schwannomas are uncommon tumors that mimic the clinical features of many other neurotologic conditions. A high index of suspicion and precise imaging are often required to detect these tumors. Surgical treatment is indicated for specific indications and will be needed in the minority of patients with this disorder. The classification system that we propose is helpful in both the diagnosis and the management of these tumors.

Avoiding misdiagnosis in ceruminous gland tumours.

Ceruminous gland tumours are infrequent lesions of the external auditory canal (EAC). Controversy still exists about nomenclature, classification, tissue of origin, and accurate diagnosis of these tumours. We present three cases of ceruminous gland tumours, including benign eccrine cylindroma, ceruminous adenoma, and adenoid cystic carcinoma. Superficial biopsy led to an initial erroneous diagnosis of adenocarcinoma in the latter. All cases were positive for cytokeratin and S-100 protein, supporting a ceruminous gland origin. The benign eccrine cylindroma should be included in the ceruminous gland tumour classification. A wide excisional biopsy should be performed in every EAC lesion. The terms 'ceruminoma' and 'cylindroma' should be avoided.

[Clinical classification of benign ENT tumor]. / Klinicheskaia klassfikatsiia dobrokachestvennykh opukholei LOR-organov.

The proposed clinical classification of benign ENT tumors is based on division of anatomic regions (the ear, nose, pharynx, larynx) into sections and fragments and allows to assess the spread of any ENT tumor. Moreover, it agrees with TNM classification. The symbols "T" and R are used which denote four variants (T1-4) or R1-4 for the tumor and recurrence, respectively.

Recurrent glomus tumor of the pinna: report of a case.

We describe a rare case of a glomus tumor of the pinna. The lesion produced a brief but sharp pain that occurred spontaneously, intermittently, and upon tactile stimulation. Surgical excision with wide margins was successful. We believe this to be only the third case of a glomus tumor of the auricle that has been reported in the literature.

Squamous cell carcinoma of the external auditory canal: an evaluation of a staging system.

OBJECTIVE: The study was conducted to review a staging system proposed by the University of Pittsburgh for temporal bone cancer and to evaluate survival status according to stage, treatment, and certain prognostic factors. STUDY DESIGN: The study was a retrospective case review. SETTING: The study was conducted at a tertiary care medical center and specialty hospital. PATIENTS: Thirty-two patients with primary squamous cell carcinoma of the external auditory canal were studied. INTERVENTION: All patients underwent surgery of the temporal bone. Radiotherapy was given depending on tumor stage and histopathologic findings. MAIN OUTCOME MEASURES: The 2-year survival rates of patients undergoing surgical resection with or without adjuvant radiotherapy. RESULTS: The 2-year survival rates for primary squamous cell carcinoma of the temporal bone were as follows: T1 lesions 100%, T2 80%, T3 50%, and T4 7%. Survival for T3 tumors was 75% with postoperative radiotherapy, compared with 0% with surgery alone. CONCLUSIONS: The 2-year survival data directly correlated with the staging system. The use of adjuvant radiotherapy increased survival rate in patients with a T3 lesion.

Eustachian tube teratoma and its terminological correctness.

Teratomas are germ cell tumours usually found in the young and are characteristically composed of tissue foreign to the place where they arise. Two teratomas of the middle ear were first described in 1866 and since then, and to the best of our knowledge, 19 additional cases have been reported in the literature under different terms such as hairy congenital polyps, epidermoid cysts, dermoid cysts, hamartoma, cutaneous teratoma and teratomas. The difficulty in classifying germ cell tumours may explain the different terminologies encountered in the literature. The authors describe a case of teratoma of the eustachian tube presenting as a mass in the middle and external ear of a 10-week-old girl. Using this case, a review of the literature is performed in light of the new classification of germ cell tumours proposed by the World Health Organization (WHO). It is of note that most of the teratomas that present in the middle ear arise from, or involve, the eustachian tube.

Endolymphatic sac tumours.

This review article surveys clinical and pathological literature on endolymphatic sac tumours (ELST) and summarizes characteristics that describe the entity. ELST are rare neuroectodermal neoplasms in the petrous bone, originating from inner ear structures. They can be encountered sporadically or in von Hippel-Lindau disease. The most prominent symptom is sensorineural deafness. Historically, nomenclature of invasive adenoid tumours in the petrous bone has been divergent, the term papillary adenocarcinoma used most frequently. Histologically, they have a follicular or papillary and adenoid pattern that can be easily confused with various other neoplastic conditions including metastatic carcinoma. It remains to be verified whether similar tumours (papillary adenocarcinomas) can originate from the middle ear. Middle ear adenomas have a similar appearance but probably originate from neural crest cells in the middle ear. ELST can express a variety of epitopes (including cytokeratin and neuroectodermal markers) which can be detected immunohistochemically. In cases in von Hippel-Lindau disease the cerebello-pontine angle should be included in routine radiological examinations to detect ELST before the tumours lead to deafness. In apparently sporadic cases of ELST, genetic testing for von Hippel-Lindau disease should be considered. Correct distinction of ELST from metastatic carcinoma prevents futile searches for unknown primary tumours.