[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.

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.

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.

Management and outcome of middle ear adenomatous neuroendocrine tumours: A systematic review.

Middle ear adenomatous neuroendocrine tumours (MEANTs) are rare, unpredictable tumours. Although most MEANTs are characterized by a benign biological behaviour and indolent growth pattern, some studies have reported locally invasive and metastastic disease. Currently, the optimal management strategy for MEANTs remains subject of debate. The aim of this study is to review the literature on MEANTs with focus on its clinical characteristics, treatment strategies and outcome. A systematic review was conducted using PubMed, Embase and Cochrane databases. A total of 111 studies comprising 198 patients with MEANT were included. Treatment modalities comprised surgery (90%), surgery with adjuvant radiotherapy (9%) and palliative (chemo)radiotherapy in (1%). Local recurrence was observed in 25% of the patients and 7% of the patients developed metastasis, over a median period of 5.7 years (range 7 months - 32 years). Twelve of 13 patients (92%) who developed metastases had a local recurrence. Four patients (2%) died of MEANT: three due to distant metastases and one due to extensive local recurrence. Reliable histopathologic predictors of outcome could not be identified. These findings indicate that the clinical presentations of MEANT vary substantially, the overall recurrence rate is considerable and initial local tumour control is paramount. Because of the unpredictable clinical course, prolonged follow-up is warranted.

Management of vestibular dysfunction and hearing loss in intralabyrinthine schwannomas.

PURPOSE: Intralabyrinthine schwannomas (ILS) are rare, benign, slow-growing tumors arising from schwann cells of the cochlear or vestibular nerves within the bony labyrinth. This study provides insight into the management of this rare tumor through a large case series. MATERIALS AND METHODS: After Institutional Review Board approval, a retrospective chart review was performed of all ILS patients treated at our institution between 2007 and 2019. RESULTS: 20 patients (9 male, 11 female) with ILS were managed at our institution. The right ear was affected in 9 patients (45%) and the left in 11 (55%). Subjective hearing loss was endorsed by all 20 patients. Average pure tone average at presentation was 72 dB nHL. Nine tumors (45%) were intravestibular, 6 (30%) were intracochlear, 4 (20%) were transmodiolar and 1 (5%) was intravestibulocochlear. Hearings aids were used in 3 patients (15%), BiCROS in 2 (10%), CI in 2 (10%), and bone conduction implant in 1 (5%). Vestibular rehabilitation was pursued in 5 patients. Surgical excision was performed for one patient (5%) via translabyrinthine approach due to intractable vertigo. No patients received radiotherapy or intratympanic gentamicin injections. CONCLUSION: ILS presents a diagnostic and management challenge given the similarity of symptoms with other disorders and limited treatment options. Hearing loss may be managed on a case-by-case basis according to patient symptoms while vestibular loss may be mitigated with vestibular therapy. Surgical excision may be considered in patients with intractable vertigo, severe hearing loss with concurrent CI placement, or in other case-by-case situations.

Survival in Middle Ear Malignancy: A Population-Based Analysis Based on the SEER Database.

OBJECTIVE: The purpose of this study was to investigate the clinical features and survival outcomes of patients with middle ear malignancies at a population level. STUDY DESIGN: Retrospective cohort study with data from a national database. SETTING: National database of middle ear malignancy. METHODS: Records of patients diagnosed with a middle ear malignancy from 1973 to 2016 were extracted from the SEER database (Surveillance, Epidemiology, and End Results). SPSS (version 27; IBM) was used to conduct 5-year survival analysis. RESULTS: The average survival for all 431 patients was 61.4 months. Five-year disease-specific survival for squamous cell carcinoma (SCCA), adenocarcinoma, other carcinoma, and noncarcinoma subtypes varied significantly at 54.6%, 82.1%, 71.8%, and 82.6%, respectively (P < .0001). There was an improved 5-year survival for patients with adenocarcinoma who received surgery versus those who did not (91.7% vs 65.1%; P = .023, log-rank). Five-year disease-specific survival was significantly better in patients aged <55 years (mean ± SD, 77.8% ± 0.39%) as compared with those >70 years (55.1% ± 5.1%) and those aged 55 to 69 years (60.2% ± 4.9%; P < .01 and P < .001, respectively, log-rank). Patients with SCCA were significantly older than those with adenocarcinoma (P < .0001). Noncarcinoma subtypes were more likely to present with local disease, as opposed to regional or distant disease, when compared with SCCA (P = .0027). CONCLUSION: Prognosis and treatment outcomes for primary middle ear malignancies depend on histologic subtype and age at diagnosis. The noncarcinoma and adenocarcinoma subtypes carry the best prognoses. Patients with adenocarcinoma were most likely to benefit from surgery.

Concurrent chemoradiation in locally advanced primary middle ear lymphoepithelial carcinoma: an effective treatment modality case report.

BACKGROUND: Definitive treatment of primary middle ear lymphoepithelial carcinoma (LEC) is not well established owing to the rarity of this disease entity. We report a case of locally advanced primary middle ear LEC treated with concurrent chemoradiation, with good oncologic outcomes. CASE PRESENTATION: A 46 year-old female of Cantonese (Southern Chinese) descent presented with a four-month history of left sided hearing loss and non-pulsatile tinnitus, associated with progressive ipsilateral facial weakness. She had a left facial palsy (House-Brackmann 2) which then deteriorated to complete palsy over 2 weeks. Otoscopic examination of the left ear revealed a red-hued mass replacing the tympanic membrane. There was no cervical lymphadenopathy. Fibreoptic nasoendoscopy was unremarkable. Pure tone audiometry revealed profound mixed left hearing loss with type B impedance. Computed tomography of the temporal bone showed an ill-defined left middle ear mass with erosion of the malleus, tegmen tympani and facial canal. Magnetic Resonance Imaging showed an avidly enhancing lesion involving the dura of the left middle cranial fossa, tympanic and labyrinthine portions of the facial nerve. This mass extended into the apex of the left internal acoustic canal and basal turn of the cochlea. Histopathology confirmed EBV-positive primary middle ear LEC. Concurrent chemoradiation comprising 70Gy of intensity-modulated radiation therapy and 3 cycles of concurrent Cisplatin based chemotherapy over a 6 week period was administered. The patient achieved near-complete disease resolution on 3 month post-treatment imaging. Serum EBV DNA titres declined to undetectable levels and the patient is disease-free at 18 months post-diagnosis. DISCUSSION AND CONCLUSION: Concurrent chemoradiation with curative intent may be a viable treatment option for locally advanced middle ear LEC not amenable to surgical resection due to expected surgical morbidity. It confers good oncologic outcomes that mimic the response in other head and neck EBV-related lymphoepithelial carcinomas.

Multi-institutional Survey of Squamous Cell Carcinoma of the External Auditory Canal in Japan.

OBJECTIVES: This study aimed to evaluate the efficacy of chemoradiotherapy (CRT) for patients with advanced cancer of the external auditory canal (EAC) by analyzing the outcome of the patients. METHODS: This is a multi-institutional retrospective survey, and we reviewed the medical records of the subjects. A total of 181 patients with tumor (T)3 or T4 tumor in 17 institutions were enrolled. Further analysis was performed for 74 patients who underwent CRT under curative intent. RESULTS: Overall 5-year survival rates of the patients who underwent CRT (n = 74) were 54.6%. Those of the patients who underwent CRT with modified TPF (docetaxel, cisplatin [CDDP], and 5-fluorouracil) regimen (n = 50) and CRT with CDDP regimens (n = 24) were 64.4% and 36.7%, respectively. Significant differences were observed between these two groups. CONCLUSION: Given the tendency that head and neck surgeons prefer CRT for advanced larger cancer of the EAC, CRT for advanced EAC cancer using the modified TPF regimen showed good clinical outcomes. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E870-E874, 2021.

Leptomeningeal Relapse of Embryonal Rhabdomyosarcoma after 15 years.

Aim Rhabdomyosarcoma (RMS) is the most common malignant soft tissue tumour of childhood. We present the case of a late relapse of RMS to the leptomeninges after 15 years. Methods A 20 year old male presented with a 3 week history of headaches and nausea. He previously had RMS of his right ear diagnosed at age 5 years which was treated with concurrent chemoradiotherapy. An MRI Brain and Spine confirmed extensive leptomeningeal disease and CSF analysis confirmed the presence of recurrent embryonal RMS. Results He completed two cycles of cyclophosphamide and topotecan followed by 45Gy/25Fr of craniospinal radiotherapy. Conclusion Late relapses beyond five years can be seen in up to 9% of patients, however very late recurrences (>10 years) are exceedingly rare. Molecular based methods such as gene expression profiling can aid risk stratification and survivorship clinics may become increasingly useful in following patients with high risk features.

Clinical features and treatment of endolymphatic sac tumor.

Background: Endolymphatic sac tumor (ELST) is an extremely rare disease that does not have established clinical guidelines.Objective: To provide guidance for the diagnosis and management of ELSTs.Methods: We retrospectively reviewed the medical records of patients who were suspected to have a lesion in the location of endolymphatic sac. Seven patients were included. Histopathologic diagnosis confirmed five ELSTs and two other diseases.Results: All of the enrolled patients had hearing impairment. Facial palsy was found in four out of five patients with ELST, whereas none of the patients in other disease group showed facial palsy. CT and MRI findings were consistent with those in previous literatures; however, angiographic findings were not consistent with those previously reported. All patients underwent surgery. Recurrence occurred in two patients with ELST, although there was no visible remnant tumor after the operation. These patients were treated with stereotactic radiosurgery.Conclusion: Hearing impairment and facial palsy were representative symptoms of ELST. All ELSTs showed 'salt and pepper' signal in MRI T1-weighted images, and smaller tumors could have blood supply from AICA in the radiologic study. Complete surgical resection with optional stereotactic radiosurgery should be considered in ELST.HighlightsHearing impairment and facial weakness are remarkable symptoms of ELST compared to other diseases that invade the endolymphatic sac.All ELSTs showed 'salt and pepper' signal in MRI T1-weighted images.ELST less than 3 cm in diameter could have blood supply from AICA.A complete surgical excision with optional stereotactic radiosurgery is required.

[A rare pathology of the petrous part of the temporal bone: adenoma of the middle ear]. / Eine seltene Pathologie des Felsenbeins: Das Adenom des Mittelohres.

Adenomas are very rare tumors of the middle ear. They are benign neoplasms originating from the glandular components of the mucous membrane of the middle ear. The middle ear adenoma was first described by Hyams and Michaels in 1976, which was named an adenomatous tumor. This article reports the case of a 50-year-old female patient, who presented with recurrent right-sided dull otalgia and pulse synchronous tinnitis, which began 1 year prior to presentation, with the suspected diagnosis of a glomus tympanicum tumor. Following the otorhinolaryngological examination and imaging an unclear mesotympanal space-occupying lesion was detected. A transmeatal endoscopic complete removal of the tumor was carried out. The histopathological investigations enabled the diagnosis of an adenoma of the middle ear. Adenomas are a rare differential diagnosis of tumors of the middle ear. In cases with a suitable localization an adequate exposure and removal of this rare tumor can be achieved by a transmeatal endoscopic access.

Meta-analysis does not support routine traditional nuclear medicine studies for malignant otitis.

OBJECTIVES: The role of traditional nuclear medicine studies in the management of malignant otitis externa (MOE) is unclear and there are ongoing debates regarding their diagnostic value. The authors perform a systematic review and meta-analysis to assess the sensitivity and specificity of traditional nuclear medicine studies in the diagnosis of MOE. METHODS: In accordance with PRISMA guidelines, a query of the Medline, Embase, Web of Science, and Cochrane databases was undertaken. The primary outcomes of interest were the sensitivity and specificity of traditional nuclear medicine studies to detect MOE. RESULTS: Of the initial 1317 hits from the four databases, 20 articles with a combined 608 patients were ultimately included in the review. The pooled sensitivities for Technetium-99 and Gallium-67 were 85.1% (95% CI, 72.0-98.1%) and 71.2% (95% CI, 55.1-87.3%) respectively. The available evidence suggested poor specificity of these modalities, but was insufficient for meta-analysis. Neither modality was shown to be effective in the assessment of disease resolution. CONCLUSION: The sensitivities of Technetium-99 and Gallium-67 to detect MOE are less favorable than was initially thought. Given this finding and their poor specificity, lack of anatomic resolution, unproven ability to detect disease resolution and variable availability, this review does not support the routine use of these studies in the management of MOE. LEVEL OF EVIDENCE: N/A Laryngoscope, 130:1812-1816, 2020.

Epidermal Carcinoma of the Conchal Bowl: Creation of a Multidisciplinary Pathway Approach.

BACKGROUND: Malignant neoplasms of the auricle make up 6% of all skin cancers. Management of cutaneous neoplasms of the conchal bowl presents a unique challenge in visualizing and defining margins that may extend into the external auditory canal (EAC). OBJECTIVES: The objective of this study was to create a multidisciplinary pathway for cutaneous carcinoma of the conchal bowl extending into the EAC. METHODS: We present a series of patients that were referred to dermatology or otolaryngology, with cutaneous neoplasms arising in the conchal bowl. A consensus approach from otolaryngology and dermatology, for evaluation and treatment, was created based on evaluation of these cases, and review of the otolaryngology and dermatology literature, in collaboration between the two specialties. RESULTS: Initial evaluation should be done by both dermatology and otolaryngology, with otomicroscopic evaluation of the canal. Imaging is recommended for lesions that approach the EAC, for bony and soft tissue spread. Excision of the tumor with Mohs micrographic surgery to achieve clearance in the conchal bowl should be performed initially. If extension into the external auditory meatus is present, otolaryngology would proceed with en bloc resection. Repair is dictated by the defect, with both specialties involved in follow-up. CONCLUSIONS: In collaboration between dermatology and otolaryngology, and following review of the literature, a pathway was created to manage skin cancer of the conchal bowl. This resulted in a stepwise approach for evaluation and management, ensuring that patients have a streamlined pathway for the treatment of these lesions.

National Analysis of Patients With External Ear Melanoma in the United States.

BACKGROUND: External ear melanoma (EEM) is a rare condition with controversies in the literature. We analyzed patients with EEM in the United States compared to other head and neck melanomas (OHNMs). METHODS: The National Cancer Database (NCDB) was used to select patients with head and neck melanoma from January 1, 2004 to December 31, 2015. Mann-Whitney and χ tests were used to estimate statistical significance, and multivariate logistic regression to identify independent associations adjusted for confounders. RESULTS: A total of 137,233 patients met the study criteria. Among them, 16,991 (12.4%) had EEM and 120,242 (87.6%) had OHNM. For patients with EEM, the mean (standard deviation) age was 66.26 (15.798) years. Most of the patients with EEM were men (85.5%), insured by Medicare (52.4%), and treated in Academic/Research Programs (47.7%) or Comprehensive Community Cancer Programs (32.3%). Most of the EEM tumors had invasive behavior (68.0%) were Stages 0 (30.3%) or I (40.3%), and were without ulceration (76.9%). Mean time to receive any treatment was 14.1 days for EEM compared with 14.6 days for OHNM (P < 0.001). We noticed a greater proportion of EEM in men (14.8%; adjusted odds ratio [aOR] 2.72 [2.605-2.852]; P < 0.001) compared to women (6.22%; reference). EEM was an independent factor for tumor Stage I (14.47%; aOR 1.61 [1.101-1.224], P < 0.001) and invasive behavior (13.86%; aOR 1.268 [1.15-1.389]; P < 0.001) compared to OHNM. CONCLUSION: EEM was associated with higher odds of invasive behavior compared to OHNM. Furthermore, men were found to have a higher likelihood to develop EEM compared to women.

Malignant otitis externa, an increasing burden in the twenty-first century: review of cases in a UK teaching hospital, with a proposed algorithm for diagnosis and management.

BACKGROUND: Malignant otitis externa is a potentially fatal infection of the skull base. With an ageing population and increasing prevalence of diabetes, the incidence of malignant otitis externa in the British population is rising. To date, there remain no accepted diagnostic criteria, few prognostic indicators and no consensus treatment pathways. METHOD: A prospective case series was conducted at a tertiary referral teaching hospital. RESULTS: A cohort of susceptible individuals predominates (elderly, male, with immunosuppression and diabetes), with 25 per cent reporting a preceding incident. Otorrhoea, otalgia and canal granulation were the commonest presenting features, alongside positive pseudomonas cultures. No clear markers for predicating disease severity were isolated; however, a high initial haemoglobin A1c level demonstrated a significant moderately positive correlation with length of treatment. CONCLUSION: A treatment pathway designed to provide a standardised approach to investigation and treatment is proposed, which aims to increase earlier diagnosis, streamline care and facilitate the development of best practice.

Effectiveness of chemoradiotherapy for radiation-induced bilateral external auditory canal cancer: A case report and literature review.

BACKGROUND: Radiation-induced bilateral external auditory canal cancer is an extremely rare disease that has yet to be fully characterized in the clinical literature. METHODS: Herein, we present a case study of a 75-year-old man with radiation-induced bilateral external auditory canal cancer. The patient's medical history included left maxillary cancer that had been treated with chemoradiation 19 years earlier and local recurrence with total maxillectomy 10 years earlier. Intracavitary radiation was delivered to the site of postoperative recurrence 8 years before the current presentation. The patient declined radical surgery for the external auditory canal cancer at this time, and a customized combined modality regimen was thus administered. RESULTS: There was no recurrence of cancer for 22 months, to date, after completing chemoradiotherapy. CONCLUSIONS: Our finding that radiotherapy can be successfully used for radiation-induced cancer indicates that chemoradiotherapy may be a useful strategy for treating this type of malignancy.

Bilateral radiation-induced squamous cell carcinomas of the external auditory canal 30 years after radiotherapy for a pituitary adenoma: a case report.

INTRODUCTION: The most important long-term effect of radiotherapy is induction of secondary cancers. A rare radiation-induced tumor is a squamous cell carcinoma of the external auditory canal. Case reports have been described in Asian patients after radiotherapy for nasopharyngeal cancer. CASE DESCRIPTION: We describe an unusual case report of a 53-year-old Caucasian man who developed squamous cell carcinomas of the right and left external auditory canal, respectively 29 and 30 years after radiotherapy for a pituitary adenoma. CONCLUSION: In young patients with benign tumors, we should always evaluate whether the benefits of radiotherapy outweigh the risks and side effects. After radiotherapy, screening programs should be developed since early detection improves salvage treatment of these secondary and often morbid tumors.