Blue laser for the exclusive endoscopic transcanal approach to middle ear paraganglioma.

BACKGROUND: The management of glomus tympanicum tumours can be challenging. Blue laser coagulation may improve bleeding control thus facilitating an endoscopic transcanal excision. The objective of this presentation is to illustrate the authors' experience using this novel tool. METHODS: Case report of a patient that underwent exclusive endoscopic transcanal blue laser surgery of a class A2 glomus tympanicum tumour in a tertiary referral center. CONCLUSION: The present study provides evidence of the safety and efficacy of endoscopic blue laser surgery, for the minimally invasive treatment of early-stage glomus tympanicum tumours.

[Unusual presentation of a mast cell tumor in the ear canal of a cat]. / Ungewöhnlicher Mastzelltumor im Gehörgang einer Katze.

A 12-year-old male neutered European Shorthair cat was presented for pruritus in the right ear region, bleeding from ear canal and a suspected polyp-like mass in its lumen.After the diagnostic imaging a biopsy of the mass was taken and submitted for histopathological evaluation. Histopathologic examination led to the diagnosis of low grade mast cell tumor. The subsequent staging examinations included ultrasonography of the liver and spleen as well as a complete blood count. Total ear canal ablation was performed on the same day, and the removed ear canal was again submitted for histopathologic evaluation of the surgical margins. The excision incision margins were free from infiltrating tumor cells. The cat was euthanised 14 months after the surgery. It is unknown whether the reasons for this were associated to metastatic spread of the initial mast cell tumor.A mast cell tumor in the ear canal is an unusual and rare finding, however it should be included in the list of differential diagnoses for ear canal tumors.

Temporal bone management in external and middle ear carcinoma.

PURPOSE OF REVIEW: The purpose of this review is to outline the temporal bone management of external and middle ear carcinoma. The review will outline the current evidence involved in deciding which surgical approach to take, as well as new advances in auditory rehabilitation and immunotherapy. RECENT FINDINGS: Traditional surgical approaches include lateral temporal bone resection, subtotal temporal bone resection and total temporal bone resection. They can also involve parotidectomy and neck dissection depending on extension of disease into these areas. Options for auditory rehabilitation include osseointegrated hearing aids, transcutaneous bone-conduction implants, and active middle ear implants. Recent advances in immunotherapy have included the use of anti-PD-1 monoclonal antibodies. SUMMARY: The mainstay of management of temporal bone disease involves surgical resection. Early-stage tumours classified according to the Pittsburgh staging tool can often be treated with lateral temporal bone resection, whereas late-stage tumours might need subtotal or total temporal bone resection. Parotidectomy and neck dissection might also be indicated if there is a risk of occult regional disease. Recent advances in immunotherapy have been promising, particularly around anti-PD-1 inhibitors. However, larger clinical trials will be required to test the extent of efficacy, particularly around combination use with surgery.

Questioning the value of stalk drilling after external auditory canal osteoma excision: case series, literature review, and meta-analysis.

OBJECTIVES: To question the value of drilling the site of the stalk ("insertion site" or "stalk" drilling) of a pedunculated external auditory canal osteoma (EACO) in reducing recurrence. DATA SOURCES: A retrospective medical chart review of all patients treated for EACO in one tertiary medical center, a systematic literature review using Medline via "PubMed", "Embase", and "Google scholar" search, and a meta-analysis of the proportion for recurrence of EACO with and without drilling. RESULTS: The local cohort included 19 patients and the EACO origin was the anterior EAC wall in 42% and the superior EAC wall in 26%. The most common presenting symptoms were aural fullness and impacted cerumen (53% each), followed by conductive hearing loss (42%). All patients underwent post-excision canaloplasty, and one sustained EACO recurrence. Six studies suitable for analysis were identified (63 EACOs). Hearing loss, aural fullness, otalgia, and cerumen impaction were the most common clinical presentations. The most common EACO insertion site was the anterior EAC wall (37.5%), followed by the superior EAC and posterior walls (25% each). The inferior EAC wall was least affected (12.5%). There was no significant difference in recurrence between EACOs whose stalk insertions were drilled (proportion 0.09, 95% confidence interval [CI] 0.01-0.22) to the ones whose insertion was not drilled (proportion 0.05, 95% CI 0.00-0.17). The overall recurrence proportion was 0.07 (95% confidence interval 0.02-0.15). CONCLUSION: EACO insertion site drilling does not reduce recurrence and should be avoided in the absence of a definite pedicle projecting to the EAC lumen.

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.

Transcanal Endoscopic Management of Middle Ear Paragangliomas.

OBJECTIVE: To evaluate the clinical and audiological outcomes of transcanal endoscopic resection of middle ear paragangliomas. STUDY DESIGN: Retrospective multicenter study. SETTING: Tertiary referral center and private otology clinic. PATIENTS: Patients who underwent transcanal endoscopic surgery between January 2015 and September 2020. INTERVENTIONS: Transcanal endocope-assisted resection of middle ear paragangliomas. MAIN OUTCOME MEASURES: Demographic data. RESULTS: Twenty-three patients (2 men, 21 women) with a mean (standard deviation [SD]) age of 50.5 (11.8) years and stage 1 or 2 disease were included in the study. The mean follow-up time was 2.7 years (range, 1-5 yr). Preoperatively, the mean (SD) air-conduction threshold was 33.8 (17.9) dB, and the mean (SD) air-bone gap was 13.1 (13.9) dB. Postoperatively, the mean (SD) air-conduction threshold was 25.7 (10.2) dB, the mean (SD) air-bone gap was 6.3 (6.1) dB. The mean (SD) hospital stay was 27.7 (9.9) hours. No tumor regrowth was detected on magnetic resonance imaging during postoperative follow-up. CONCLUSIONS: Endoscopic transcanal tumor resection is effective and feasible in the treatment of stage 1 and 2 tumors and is associated with short operative time, low risk of perioperative and postoperative complications, and rapid discharge.

Surgical Management of Endolymphatic Sac Tumor: A Systematic Review and Meta-Analysis.

The aim of our study was to report rates of facial nerve palsy and residual tumor following surgical intervention and subsequent tumor recurrence in patients with endolymphatic sac tumors. A systematic literature review of preoperative assessment and surgical management is also included. Studies including patient/s affected by sporadic or von Hippel-Lindau disease related endolymphatic sac tumors, reporting levels of facial nerve function, residual and recurrence pathology following a surgical procedure, were considered. Data were combined for proportional meta-analysis, and the selected studies' methodological quality was also evaluated. Overall 34 papers, including 202 subjects (209 cases of endolymphatic sac tumors) were analyzed. Pooled proportion rate (95% CI) of overall facial nerve palsy was 39.7% (28.2-51.9) and residual tumor was 16.5% (10.3-23.7) after surgical procedure. Pooled proportion rate (95% CI) of tumor recurrence was 14.0% (9.7-19.3) during a mean follow-up period of 49.7 months (8-136). Our results showed that preoperative facial nerve function is impaired in almost 30% of patients with endolymphatic sac tumors. Surgical management of endolymphatic sac tumor may cause a worsening of facial nerve function in a low percentage of treated subjects. Residual and/or recurrence of endolymphatic sac tumors are not rare events, and follow-up strategies should be designed accordingly.

The Diagnosis and Management of Advanced Endolymphatic Sac Tumor.

Endolymphatic sac tumor (ELST) is a group of low-grade malignant tumors originating from the endolymphatic sac of the inner ear. It is rare in the clinic and has the biological characteristics of slow growth and local aggression. Due to the lack of specificity in the clinical manifestations of patients with ELST, many cases have entered the advanced stage at the time of diagnosis. However, there are still great challenges in the treatment of advanced ELSTs. Here, the authors describe a case of advanced ELST, which relapsed after 2 operations. This time, the authors chose the transotic approach for tumor resection, which achieved the goal of complete resection of the tumor, and the patient recovered smoothly after surgery. There were no surgical complications and no tumor recurrence after the follow-up. Through literature review and our own experience, the authors suggest that complete surgical resection is the first choice for both primary and recurrent advanced ELSTs. The choice of a reasonable surgical approach is the key to ensuring complete resection of the tumor, while preoperative angiography and embolization, fine treatment of important structures during surgery, and postoperative long-term follow-up are equally important for patients with advanced ELST to obtain a good prognosis.

Clinical outcome and prognostic factors in adenoid cystic carcinoma of the external auditory canal: proposal for a refined T-stage classification system.

PURPOSE: Although adenoid cystic carcinoma (ACC) of the external auditory canal (EAC) has a different pathophysiology from squamous cell carcinoma, the same staging system is used. The aim of this study was to propose a refined staging system, which is more suitable for ACC of the EAC. METHODS: A total of 25 patients who were diagnosed with ACC of the EAC were reviewed. The modified Pittsburgh staging system (mPSS) that is universally used for temporal bone malignancy was refined for ACC (rPSS). The limited (< 0.5 cm) lateral soft tissue involvement was classified as T1 and extensive (≥ 0.5 cm) lateral soft tissue involvement as T2. The disease-free survival rate (DFSR) was assessed in the patients who underwent surgical treatment according to two staging systems; mPSS and rPSS. RESULTS: When staging using mPSS, most patients (96.0%, n = 24) were classified as T4. However, when rPSS was used, T1, T2, T3, and T4 stage occupied 36.0% (n = 9), 40.0% (n = 10), 12.0% (n = 3), 12.0% (n = 3), respectively. There was no difference in DFSR according to the T stage using mPSS (p = 0.466). However, when rPSS was used, the DFSR showed significant correlation with the T stage (p = 0.032). CONCLUSIONS: Clinical T stage of mPSS was not sufficient to predict survival rate in ACC of the EAC, and we propose that the information on the lateral soft tissue involvement needs to be added to the existing staging system.

Middle Ear Teratoma: Clinical and Imaging Features.

BACKGROUND: Teratoma is a true neoplasm composed of a number of different types of tissue derived from the three germinal layers but rarely occurs in the middle ear (ME). The features of middle ear teratomas (MET) have not been well described. OBJECTIVE: The objective of this study is to explore the clinical and imaging features of MET, and report 2 rare cases of MET with ear malformation that have never been reported. MATERIALS AND METHODS: The clinical, CT and MRI data of 8 patients with a pathological diagnosis of MET were collected and retrospectively mined, and 14 patients with MET reported in previous literature were also reviewed. RESULTS: ① Female, left ear predominance in MET, and the most common symptoms were otorrhea and hearing loss. ② On CT and MRI, the MET presented as an irregular soft tissue mass that was heterogeneous, with fatty tissue and involved multiple sites, and the ET and tympanum were correspondingly expanded and locally destroyed. ③ Mictotia with MET in two patients was presented, which was the first report. CONCLUSION: MET has female sex and left ear predominance. CT and MRI can be used to diagnose MET and display its extent and its relationship to the carotid canal in detail. Complete surgical excision is the definitive treatment.

Middle-ear carcinoid tumour metastasising to the parapharyngeal space and the parotid gland.

Background. Middle-ear carcinoid tumour is a rare malignant tumour with an indolent course occasionally causing regional or distant metastasis. This paper presents a case of middle-ear carcinoid tumour metastasising to the parapharyngeal space and the parotid gland 20 years after the first surgery.Case report. A 35-year-old woman who underwent multiple tympanomastoidectomies for middle-ear carcinoid presented with tumours of both the parapharyngeal space and parotid gland, detected by regular imaging. Based on the clinical course, metastatic relapse of middle-ear carcinoid was suspected. This was treated with subtotal parotidectomy with elective neck dissection (levels II and III), leading to the pathological diagnosis of carcinoid tumour. A cervico-parotid approach was selected to avoid complications associated with parapharyngeal space tumour removal. Transient facial palsy (House-Brackmann grade III) occurred, which completely recovered two months after surgery.Conclusion. Awareness of parapharyngeal space tumours possibly caused by metastasis from a middle-ear tumour is necessary.

Middle Ear Adenomatous Neuroendocrine Tumor: A Rare Entity.

Middle ear adenomatous neuroendocrine tumors (MEANT) are rare, mainly benign neoplasms and account for less than 2% of the middle and inner ear neoplasms. There are no specific radiological or clinical findings; therefore, the diagnosis is often difficult. Biopsy prior to surgery is often proposed. The definite treatment is the complete surgical resection. We present an uncommon clinical report of a MEANT, which was treated successfully with surgical excision.

Surgical management of endolymphatic sac tumor: classification, outcomes and strategy. A single institution's experience.

PURPOSE: To review the resections of endolymphatic sac tumor (ELST) and describe our experience in the surgical management of ELST. METHODS: Retrospective investigation of consecutive patients who underwent resection of ELSTs at our hospital between 1999 and 2019. The symptoms, diagnosis, surgical findings, and outcomes were analyzed to develop a tumor staging system and corresponding surgical strategy. RESULTS: Retrospective review revealed the surgical treatment of 22 ELSTs. Based on intraoperative findings of tumor extent and size, ELSTs were classified into two types. Type-I (n = 6) referred to the small tumors that were locally confined with limited invasion of semicircular canals and dura; type-II (n = 16) referred to the large tumors that presented extensive erosion of at least one anatomic structure apart from the semicircular canals and the dura around endolymphatic sac. In this case series, Type-I ELST is amenable to resection through a transmastoidal approach, and subtotal petrosectomy is appropriate for the resection of type-II ELST. Sensorineural hearing loss (SNHL) is the most commonly preoperative symptom in both two types of cases. Five type-II ELSTs experienced recurrence and underwent reoperation, whereas all type-I ELSTs did not. CONCLUSION: ELST usually results in SNHL (95%) at the time of diagnosis. The surgical strategy and prognosis of ELST resections are different between type-I and type-II: type-I ELST is amenable to transmastoidal approach with the preservation of facial nerve, whereas type-II ELST increase the surgical difficulty and the risk of recurrence, and subtotal petrosectomy is the basic requirement for the resection of type-II ELST.

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.

Reconstruction of a full-thickness upper third defect of the helix ear using a single-stage chondrocutaneous composite transposition flap.

The external ear is a crucial part of the ear's anatomy for both functional and aesthetic purposes. We present a defect after the extirpation of an invasive squamous cell carcinoma, where the final defect involved the superior third of the outer ear, missing both cartilage and skin. The regional chondrocutaneous composite transposition flap of the ipsilateral auricular concha is a one-stage technique that successfully preserves the helical rim's shape and size.

[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.

Mucosal Melanoma of Eustachian Tube: A Case Report.

Mucosal melanoma originating from the eustachian tube is very rare, and only 15 cases were reported so far. In this study, we report a case of mucosal melanoma from the eustachian tube which was surgically managed, followed by chemoradiotherapy. A 53-year-old man presented with a history of recurrent idiopathic hemotympanum and a dark red mass in the nasopharynx protruding from the eustachian tube orifice. Under an impression of mucosal melanoma from the eustachian tube, en-bloc surgical removal was performed using the infratemporal fossa approach type C combined with a transnasal endoscopic approach followed by postoperative chemoradiotherapy. However, the disease progressed to lung metastasis, and the patient died of the disease at 13 months postoperatively. The presenting case showed a poor progression despite a margin-free surgical resection followed by chemoradiotherapy. Additional trial of new treatment options is necessary to improve the poor prognosis.

Nuances in transcanal endoscopic approach to benign middle ear tumours in adult patients.

BACKGROUND AND OBJECTIVE: Minimally invasive surgery of benign middle ear tumours is possible by using the endoscope. The optimal lighting and the broadest vision it offers, allow a transcanal approach to these rare tumours. The objective of this work is to summarise its key points through a case series. MATERIALS AND METHODS: Retrospective study of benign middle ear tumours that underwent exclusive endoscopic surgery in a third-level adult university hospital between June 2018 and June 2020. Postoperative follow-up was performed by otoendoscopy and audiometry. RESULTS: Six patients underwent surgery during the study period. Five patients were female and one male, with an average age of 57.8 years (±21.9). Four tumours were in the left ear and 2 in the right ear. These included four tympanic paragangliomas (three type I and one type II), a chorda tympani neuroma, and a congenital cholesteatoma. There were no serious postoperative complications. At present, no tumour recurrence has been found in either case, with a minimum follow-up of 7 months. CONCLUSIONS: The present study adds evidence on the safety and efficacy of endoscopic transcanal ear surgery, as a minimally invasive technique, for the treatment of benign middle ear tumours confined to the tympanic cavity.