Endolymphatic sac tumor misdiagnosed as metastatic renal cell carcinoma: Pitfalls in morphology and immunohistochemistry.

Endolymphatic sac tumor (ELST) is a rare disease that originates from the endolymphatic sac system of the inner ear. Being a low-grade malignant tumor, ELST has a mild morphology and is characterized by a slow but aggressive growth. Most clinicians and pathologists are unfamiliar with this disease. ELST can be misdiagnosed as metastatic renal cancer because of the similarity in morphology and expression of nephrogenic markers such as PAX8. The presented case of a 27-year-old man revealed that observing the characteristic location and confirming the absence of renal neoplasm to rule out the possibility of metastasis are critical for obtaining an accurate final diagnosis.

[Malignant external auditory canal tumors : management of a rare tumor]. / Tumeurs malignes du conduit auditif externe : prise en charge d'une tumeur rare.

Malignant tumors of the external auditory canal are rare tumors and very often diagnosed at an advanced stage due to non-specific symptoms. The best treatment is the radical surgery, eventually followed by radiotherapy. A multidisciplinary team is essential to realize an optimal management. Prognosis remains unfavorable for advanced tumors.

Les tumeurs malignes du conduit auditif externe (CAE) sont rares et leur diagnostic est souvent retardé en raison de symptômes peu spécifiques. Le traitement de choix consiste en une chirurgie radicale, éventuellement suivie d'une radiothérapie. Une équipe multidisciplinaire est indispensable pour une prise en charge optimale. Le pronostic reste défavorable dans les tumeurs avancées.

External auditory canal sebaceous carcinoma.

The incidence of sebaceous carcinoma (SC) in the outer one-third of the external auditory canal is considered extremely rare, and only eight case reports have been published. We present a case of a male patient in his late 70s known case of hypertension on indapamide. His medical history included a postspinal tumour that had been treated with surgery and radiation more than 40 years ago and current complaints of right ear pain and purulent discharge. A right ear soft granular tissue mass was found. Complete debulking of the right ear mass was done in conjunction with middle ear exploration, moderately differentiated SC diagnosis was made based on the histopathological analysis. The patient was free of recurrence but then died of an unrelated pulmonary infection. SC should be suspected in elderly patients who present with long-term complaints of a mass with or without otalgia, and these patients should be examined for the presence of such tumours especially if they report a history of radiation. Additionally, more research is warranted to investigate the association of diuretics with SC.

Middle ear adenoma: Cytohistologic features and differential diagnosis.

Middle ear adenomas are rare, low grade glandular neoplasms with epithelial and neuroendocrine components and with varying patterns of differentiation. Due to the rarity of this tumor, there is a dearth of publications detailing the cytological features. We herein review our institution's pathological database for cytological material between 1992 and 2022 for MEA specimens and discuss possible differential diagnoses based on clinical, pathological, and cytologic data and material.

Carotid Sympathetic Plexus Schwannoma Presenting as Middle Ear and External Auditory Canal Mass.

Schwannomas in the middle ear and external auditory canal are exceedingly rare. The facial nerve, chorda tympani nerve, and Jacobson's nerve have rarely been reported as the origins of primary schwannomas in the middle ear cavity. We experienced a case of carotid sympathetic plexus (CSP) schwannoma that arose from the carotid canal and extended into the middle ear and external auditory canal. The tumor presented bone erosion of the carotid canal, and it adhered tightly to the internal carotid artery. This report represents the first documented case of a CSP schwannoma, which involved the middle ear and external auditory canal.

Systematic Review of Endolymphatic Sac Tumor Treatment and Outcomes.

OBJECTIVE: Endolymphatic sac tumors are rare neoplasms originating in the endolymphatic sac. Current literature is limited to case reports and small case series. The objective of this study was to systematically review the literature to better describe clinical presentation, treatment options, and outcomes in endolymphatic sac tumors. DATA SOURCE: PubMed, Embase, and Cochrane Library. REVIEW METHODS: A systematic review was performed following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines describing human endolymphatic sac tumors. Risk of bias was assessed using a validated critical appraisal checklist for case series. Studies without inclusion of individual patient characteristics, corresponding treatments, and outcomes were excluded. Heterogeneity of data precluded meta-analysis. RESULTS: A total of 82 studies met inclusion criteria, and 253 discrete tumors were analyzed. A total of 23.4% of patients had von Hippel-Lindau disease. Von Hippel-Lindau-associated tumors affected females to males in a 2.4:1 ratio. Patients with von Hippel-Lindau disease displayed earlier average age at diagnosis compared to the sporadic cohort. Surgery was the primary treatment modality and was performed in 88.9% of cases. Adjuvant radiation therapy was employed in 18.7% of cases; 16.2% cases recurred, and 10.6% had progression of residual disease after treatment. Mean time to recurrence or progression was 53.1 ± 52.4 months with a range of 3 to 240 months. CONCLUSION: Endolymphatic sac tumors require a high degree of suspicion for early diagnosis. Complete resection is the standard of care. No strong evidence supports routine use of adjuvant radiation therapy. Given the high rate of recurrence and wide-ranging time to recurrence, long-term follow-up is necessary.

[Neuroendocrine adenoma middle ear]. / Neiroendokrinnye adenomy srednego ukha.

Neuroendocrine adenoma (NEA) is an extremely rare pathology and accounts for less than 2% of all ear tumors. The article provides an overview of the diagnosis, classification, treatment methods and algorithm for monitoring patients with NEA of the middle ear. 6 cases of NEA of the tympanic cavity are described in detail, which were diagnosed and treated in GBUZ NIKIO named after N.N. L.I. Sverzhevsky DZM for the last 5 years. Diagnosis of NEA of the middle ear is possible when performing high-resolution multislice computed tomography of the temporal bones and magnetic resonance imaging with contrast enhancement, however, the neoplasm can be verified only after a histological examination with the determination of the immunophenotype. Differential diagnosis of NEA of the tympanic cavity with other processes of the middle ear must be carried out at each stage of the diagnostic path. Surgical treatment, depending on the volume of education, allows you to remove it completely and improve the auditory function.

Clinical presentation and outcome in cats with aural squamous cell carcinoma: a review of 25 cases (2010-2021).

CASE SERIES SUMMARY: Ear canal neoplasia is uncommon in cats. Ceruminous gland adenocarcinoma is the most frequently reported malignant neoplasm of the feline ear canal, and squamous cell carcinoma (SCC) is the most common malignant neoplasm diagnosed in the feline middle ear. However, limited information exists on the outcome of cats diagnosed with SCC of the ear canal, middle or inner ear. Therefore, the objective of this study was to describe the outcome of cats diagnosed with SCC affecting these locations. Medical records were reviewed at multiple institutions to identify cats with a definitive diagnosis of SCC. Twenty-five cats were identified. Eleven cats were treated with surgery, eight with medical management, two with coarse fractionated radiation therapy, two with a combination of coarse fractionated radiation therapy and chemotherapy, one with a combination of surgery and coarse fractionated radiation therapy, one cat with systemic chemotherapy and one cat received no treatment following diagnosis. The median survival time of cats treated with surgery was 168 days vs 85 days (P = 0.28) for those treated palliatively with either medical management, radiation therapy, chemotherapy, or a combination of radiation therapy and chemotherapy. RELEVANCE AND NOVEL INFORMATION: This case series documented that SCC of the ear canal, middle and/or internal ear is a locally aggressive tumor that carries an overall poor prognosis. The median survival time for cats treated with surgery was longer than that with any other modality, but this difference was not statistically significant.

A carcinoid tumor of the middle ear masquerading as a glomus tympanicum presenting with temporal lobe hemorrhage in a 70-year-old woman: Case report and review of the literature.

BACKGROUND: Paragangliomas in the central nervous system account for 0.6% of all head and neck neoplasms, with glomus tympanicum being the most common middle ear tumor. Carcinoid tumors are neuroendocrine tumors, representing less than 1% of neuroendocrine neoplasms in the middle ear. Misdiagnoses have been reported in the literature regarding glomus and carcinoid tumors, however, none have been in the central nervous system or middle ear. CASE DESCRIPTION: A 70-year-old female with a history of left temporal lobe tumor underwent unsuccessful resection due to intraoperative bleeding at an outside institution. However, biopsy prior to aborting the case led to the diagnosis of paraganglioma. Eight years postoperatively, the patient presented at our institution with acute confusion, aphasia, and altered mental status. Imaging revealed a 4cm left temporal intraparenchymal hematoma at the known tumor site with concern for intracranial tumor extension. Surgical resection was performed and previous symptoms resolved. Final pathology revealed a Grade II atypical carcinoid tumor with an unusually high Ki-67 of 50%. CONCLUSIONS: Carcinoid tumors of the middle ear constitute a differential diagnosis for patients presenting with temporal lobe hemorrhage. A combination of immunohistochemical staining with electron microscopy can assist in differentiating the tumor types. This atypical presentation for a carcinoid tumor in the middle ear suggests the need to consider carcinoid as the diagnosis in patients with a middle ear tumor invading into the temporal lobe and causing hemorrhage. These tumors may demonstrate an unusually high Ki-67 rate, in which case they should be treated aggressively.

Endolymphatic sac tumor in an 8-month-old cat.

Background: The endolymphatic sac is an organ devoid of sensory receptors. It is connected with the endolymphatic compartment and contains endolymph. Endolymphatic sac tumor (ELST) is a rare neoplasm involving the middle and inner ear described in humans and dogs that does not show cellular characteristics of malignancy, but can be locally invasive and involve destruction of the temporal bone and adjacent structures. Case Description: An 8-month-old female cat was referred because of sudden onset of vestibular signs starting 3 days prior to referral. On clinical examination, the patient showed depression, right head tilt, left-sided facial paralysis, and horizontal nystagmus with fast phase to the left. The magnetic resonance images showed a voluminous extra-axial lesion, with irregular morphology and well-defined margins, with intracranial extension in the region of the pons, rostral medulla oblongata, cerebellar vermis, floccule, and left cerebellar hemisphere. Due to progressive clinical deterioration, the cat was euthanized 2 weeks later. A necropsy was then performed and histological samples were taken. The necropsy revealed the presence of a voluminous dark red irregular mass extending from the tympanic bulla to the posterior cranial fossa following the left glossopharyngeal nerve. The histopathological exam of the extra-axial lesion featured a nonencapsulated, moderately cellular, rather loose, proliferation of cuboidal to columnar epithelium breaching through chunks of an otherwise normal appearing dura mater and invading some cranial nerves. Sections of the cerebellum and brainstem revealed moderate, focal, impingement of the parenchyma with a very mild extension of the proliferating cells into the ventral left side of the medulla oblongata. Based on these histological characteristics, the lesion was defined as ELST, a rare neoplasm described in human beings and with two reports in dogs. Conclusion: To our knowledge, this is the first report describing an ELST in a cat.

Symptoms and clinical features in patients affected by endolymphatic sac tumor: a systematic review and meta-analysis.

PURPOSE: Most ELST data in the literature are case studies or limited to small cohorts (< 16 patients). We evaluated the main clinical signs observed at endolymphatic sac tumor (ELST) diagnosis in patients with or without Von Hippel-Lindau disease. METHODS: We conducted a comprehensive literature search in PubMed, Scopus, and Web of Science. We included studies with at least 1 patient, of any age, affected by sporadic or VHL-related ELSTs reporting levels of hearing loss and facial nerve function and a comprehensive description of presenting symptoms at ELST diagnosis. We combined data for proportional meta-analysis. p values of 0.05 were considered statistically significant. Methodological quality was evaluated. Analyses were performed with MedCalc 14.8.1 software. RESULTS: A total of 26 studies, including 113 patients and 118 cases of ELSTs were included. Pooled proportion rates (95% CI) of overall hearing loss was 88.7%, (82.4-93.4), severe hearing loss was 21.6% (12.8-32.1) profound hearing loss was 39.8% (28.7-51.5), vertigo/imbalance was 42.0% (33.8-50.5), tinnitus was 61.8% (53.4-69.8) and facial nerve palsy was 30.6% (23.2-38.9). Generally, symptoms were homogeneous or moderately heterogeneous among included studies. CONCLUSION: This is the first systematic review of clinical presentations at ELST diagnosis. The most serious clinical events include profound hearing loss and facial impairment. Fluctuating hearing loss, tinnitus and vertigo are frequently reported and may confound correct and prompt ELST diagnosis.

Treatment Strategies for Malignancies of the External Auditory Canal.

OPINION STATEMENT: Malignant tumors of the external auditory canal (EAC) are rare tumors in the head and neck. Delayed diagnosis is not uncommon because the symptoms of early tumors are nonspecific. Various surgical and oncological treatment modalities have been reported. Decision-making depends on pathological feature and stage of the lesions, patient's general condition and preference, and physician's experience and skill. Radical surgery is widely accepted as the primary treatment of choice. Postoperative radiotherapy is used more often to improve local and regional control of the disease. Chemotherapy is usually recommended for advanced disease, residual disease, and metastasis. Prognosis is affected by multiple factors such as TNM stage, surgical margin, pathological type and differentiation of tumor, involvement of facial nerve, and so on. Although the survival rate is improved significantly over the past several decades with the development of skull base surgery, neuroradiology, anesthesiology, and oncology, it remains challenging to diagnose and treat EAC malignancies due to the rarity, the local anatomical complexity of temporal bone, and the lack of standard TNM staging system.

Ear canal and middle-ear tumors: a single-institution series of 87 patients.

BACKGROUND: Ear canal and middle ear tumors are rare and exhibit variability in histology and clinical manifestation. Surgical resection remains the treatment of choice, but individualized approach is needed to preserve function when possible. AIMS/OBJECTIVES: To review the management and outcome of ear canal and middle ear tumors at an academic referral center. MATERIALS AND METHODS: Helsinki University Hospital (HUS) patient files were searched for clinically and histologically confirmed ear canal and middle ear tumors over a 14-year period. The minimum follow-up time was 2 years. RESULTS: Eighty-seven patients with 88 tumors were identified. There were 20 (23%) benign external auditory canal (EAC), 36 (41%) benign middle ear space (MES), 29 (33%) malignant EAC, and 3 (3%) malignant MES tumors. Most (92%) tumors were managed with primary resection. Thirty-five percent of the operatively managed patients had a residual or a recurrent tumor. CONCLUSIONS AND SIGNIFICANCE: EAC and MES tumors show great diagnostic and histologic heterogeneity with need for individualized investigative and treatment approaches. In benign tumors, we advocate aggressive local surgical control without sacrificing vital structures. In malignant tumors, we recommend local surgical control with or without adjunct RT.

Endolymphatic sac tumor: single-institution series of seven cases with updated review of literature.

BACKGROUND: Endolymphatic sac tumour (ELST) is a rare low-grade locally aggressive neoplasm arising from the endolymphatic duct or sac. It presents mostly with vestibulo-cochlear symptoms either sporadically or as part of von Hippel-Lindau (VHL) syndrome. Micro-neurosurgical excision remains the cornerstone of therapy with the role of radiotherapy (RT) being controversial. This is a clinico-pathological analysis of consecutive ELST patients presenting to a single-institution in India. METHODS: Neuropathology database of a tertiary-care comprehensive cancer centre was searched electronically to identify consecutive patients with histopathological diagnosis of ELST registered at the institute over last one decade. Data regarding demographic profile, clinical presentation, histopathological features, treatment details and outcomes were retrieved from electronic medical records for this retrospective analysis. RESULTS: Electronic search identified seven unique patients with biopsy-proven ELST registered at the institute between 2009 and 2020. Median age of the study cohort was 39 years (range 24-65 years) with strong male predilection (5:2 ratio) and left-sided preponderance (71%). Most common presenting symptoms were hearing loss (86%) and earache (71%) on affected side followed by headache (43%). All patients underwent maximal safe resection at initial diagnosis and were followed-up closely with periodic surveillance imaging. Two patients underwent salvage RT using high-precision conformal techniques at recurrence/progression. CONCLUSION: ELST is a rare low-grade locally aggressive neoplasm that arises generally as part of VHL syndrome or sometimes sporadically. Gross total resection provides the best chance of cure with RT being reserved for unresectable disease, large residue, medical inoperability, or as salvage therapy for recurrent/progressive tumor.

A Case of Ceruminous Adenocarcinoma Not Otherwise Specified (NOS) in the External Auditory Canal.

External auditory canal cancer is a rare disease which can be treated by surgery or chemoradiation. The most common histological type is squamous cell carcinoma, but rare types such as adenocarcinoma have been reported and are thought to be derived from the ceruminous glands. Here, we present a case of ceruminous adenocarcinoma, not otherwise specified (NOS) in the external auditory canal. A 72-year-old woman was referred to our department with discomfort due to a mass in the external ear canal. No typical symptoms of malignancy, such as pain or bleeding, were noted at the initial examination. The patient underwent a total excision under local anesthesia as a diagnostic treatment. She was diagnosed with ceruminous adenocarcinoma, NOS based on the results of immunostaining of the excised specimen, and is currently being followed up as an outpatient. Adenocarcinoma is thought to originate from the cerumen glands of the ear canal and the lack of specific symptoms may make it difficult to differentiate it from benign tumors. Although adenocarcinoma, NOS has been reported in the head and neck region, there have been no reported cases occurring in the external ear canal, and to the best of our knowledge, this is the first report.

[Clinical features and prognostic factors for early-stage external auditory canal carcinoma].

Objective:To analyze the clinical features and the prognostic factors of early-stage external auditory canal carcinoma. Methods:Data from 36 patients with early-stage external auditory canal carcinoma（T1, T2） treated in Department of Otolaryngology, Xijing Hospital, Air Force Military Medical University from January 2008 to June 2020 were reviewed retrospectively, including clinical manifestations, surgical and treatment methods, pathological types and disease status. The relationship between survival rate and the prognostic factors was compared using Kaplan-Meier method, and the independent risk factors were analyzed by Cox proportional hazards model. Results:There were 36 patents with early-stage external auditory canal carcinoma. The common initial symptoms were otalgia（66.7%）, otorrhea（41.7%） and hearing loss（30.6%）. The most common histopathologic types were adenoid cystic carcinoma（50.0%） and squamous cell carcinoma（33.3%）. Among the patients, 21 patients（58.3%） were initially treated, 9 patients（25.0%） were treated with salvage therapy, and 6 patients（16.7%） were re-surgery after recurrence. The 5-year disease-specific survival（DSS）, disease-free survival（DFS） and relapse-free survival（RFS） were 82.3%, 64.0% and 73.0% respectively. Seven cases （19.4%） relapsed after surgery. For 5-year survival rate, the lateral temporal bone resection with superficial parotidectomy（DSS 91.7%, DFS 83.9%） is higher than the lateral temporal bone resection only（DSS 77.8%, DFS 55.6%） and sleeve resection（DSS 75.0%, DFS 56.0%）, but there was no significant difference（P>0.05）. In these patients, the postoperative radiotherapy and disease status had no significant impact on the survival rate. Additionally, there was no obvious correlation between recurrence and age, gender, stage, histopathologic types, operation methods and postoperative radiotherapy（P>0.05）. But there were significant differences between histopathologic types and DSS or DFS（P<0.05）. Multivariate regression analysis showed that histopathologic type was an independent prognostic factor for DFS. Conclusion:There are no specific clinical manifestations for early-stage external auditory canal carcinoma, such as otalgia and otorrhea. Histopathologic types have a direct impact on the patients'prognosis. Thus, individualized treatment should be applied based on pathologic findings to improve the survival rate.