Melanoma of the external auditory canal: case report and systematic literature review.

Melanoma of the external auditory canal (EAC) is particularly rare and poorly understood, with limited available data on management and survival. This systematic review aims to analyze existing data and provide insights into the management and prognosis the beginning of EAC melanoma. It is conducted using Pubmed and Scopus databases from the beginning to July 2023 and it follows the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) 2020 guidelines. Searches are performed using the search string "(melanoma) AND (external auditory canal)".The review includes a total of 30 patients diagnosed with EAC melanoma, supplemented by an additional case from the authors' clinical experience. The role of Breslow thickness as a determining factor for the choice of surgery remains inconclusive due to limited available data. Sentinel lymph node biopsy and adjuvant therapy are sparingly employed, indicating the need for standardized guidelines. Patients in the study demonstrate a 50% overall survival rate at 5 years.EAC Melanoma is a rare and aggressive malignancy with limited therapeutic guidelines. Surgical interventions, including wide local excision and lateral temporal bone resection, are the primary treatment options for patients without distant metastases.

[To assess the effectiveness of treatment methods for patients of different age groups with basal cell cancer of the skin of the nose and ears].

The relevance of the problems of diagnosis and treatment of skin cancer is currently determined not only by the high incidence rate, but by the existing difficulties in differential diagnosis and treatment with traditional methods. For localizations of basal cell skin cancer (BCSC) that are "inconvenient" for treatment, such as the external auditory canal, auricle, and wing of the nose, treatment is associated with certain difficulties and the possible appearance of a cosmetic defect, therefore, when choosing a treatment method, the anatomical features of these organs are taken into account. It has been determined that the effectiveness of treatment for primary BCSC of the nose and auricles is higher than recurrent one, and among the various treatment methods, the most effective and radical is the surgical method. The immediate results of treatment of BCSC in the form of PR by surgical method were 86.7%, which is statistically significant compared with other types of treatment (p < 0.05). Long-term treatment results with the surgical method are also higher (77%) compared to other methods, which is also statistically significant (p < 0.05).

Mohs micrographic surgery challenges and new technologies to optimize care of cutaneous malignancies of the ear.

Cutaneous malignancies affecting the ear, exacerbated by extensive ultraviolet (UV) exposure, pose intricate challenges owing to the organ's complex anatomy. This article investigates how the anatomy contributes to late-stage diagnoses and ensuing complexities in surgical interventions. Mohs Micrographic Surgery (MMS), acknowledged as the gold standard for treating most cutaneous malignancies of the ear, ensures superior margin control and cure rates. However, the ear's intricacy necessitates careful consideration of tissue availability and aesthetic outcomes. The manuscript explores new technologies like Reflectance Confocal Microscopy (RCM), Optical Coherence Tomography (OCT), High-Frequency, High-Resolution Ultrasound (HFHRUS), and Raman spectroscopy (RS). These technologies hold the promise of enhancing diagnostic accuracy and providing real-time visualization of excised tissue, thereby improving tumor margin assessments. Dermoscopy continues to be a valuable non-invasive tool for identifying malignant lesions. Staining methods in Mohs surgery are discussed, emphasizing hematoxylin and eosin (H&E) as the gold standard for evaluating tumor margins. Toluidine blue is explored for potential applications in assessing basal cell carcinomas (BCC), and immunohistochemical staining is considered for detecting proteins associated with specific malignancies. As MMS and imaging technologies advance, a thorough evaluation of their practicality, cost-effectiveness, and benefits becomes essential for enhancing surgical outcomes and patient care. The potential synergy of artificial intelligence with these innovations holds promise in revolutionizing tumor detection and improving the efficacy of cutaneous malignancy treatments.

[Facial nerve protection in surgery for rare tumors of the internal auditory canal].

Objective:To explore strategies for preserving facial nerve function during surgeries for rare tumors of the internal auditory canal. Methods:A total of 235 cases of internal auditory canal tumors treated between 2010 and 2023 were included, encompassing vestibular schwannomas, cavernous hemangiomas, meningiomas, and other rare tumors. Various data, including clinical presentations, imaging classifications, and treatment processes, were meticulously analyzed to delineate the characteristics of rare tumors and assess pre-and postoperative facial nerve function. Results:Among all internal auditory canal tumors, vestibular schwannomas accounted for 91.9%. In rare tumors, facial nerve schwannomas constituted 5.3%, cavernous hemangiomas 26.3%, meningiomas 15.8%, and arterial aneurysms 10.5%. Significantly, patients with cavernous hemangiomas displayed pronounced invasion of the facial nerve by the tumor, in contrast to other tumor types where clear boundaries with the facial nerve were maintained. During surgery, individualized approaches and strategies for facial nerve protection were implemented for different tumor types, involving intraoperative dissection, tumor excision, and facial nerve reconstruction. Conclusion:Preservation of the facial nerve is crucial in the surgical management of rare tumors of the internal auditory canal. Accurate preoperative diagnosis, appropriate timing of surgery, selective surgical approaches, and meticulous intraoperative techniques can maximize the protection of facial nerve function. Personalized treatment plans and strategies for facial nerve functional reconstruction are anticipated to enhance surgical success rates, reduce the risk of postoperative facial nerve dysfunction, and ultimately improve the quality of life for patients.

Unilateral Multifocal Inner Ear and Internal Auditory Canal or Cerebellopontine Angle Cochleovestibular Schwannomas-Genetic Analysis and Management by Surgical Resection and Cochlear Implantation.

OBJECTIVE: To describe the genetic characteristics and the management of two very rare cases of unilateral multifocal inner ear and internal auditory canal or cerebellopontine angle cochleovestibular schwannomas not being associated to full neurofibromatosis type 2-related schwannomatosis. PATIENTS: In a 29-year-old man and a 55-year-old woman with single-sided deafness multifocal unilateral cochleovestibular schwannomas were surgically resected, and hearing was rehabilitated with a cochlear implant (CI). Unaffected tissue was analyzed using next generation sequencing of the NF2 gene. Tumor tissue was analyzed using a 340-parallel sequencing gene panel. MAIN OUTCOME MEASURES: Mutations in the NF2 gene, word recognition score for monosyllables at 65 dB SPL (WRS 65 ) with CI. RESULTS: No disease-causing mutation was detected in the examined sequences in blood leucokytes. All tumor samples revealed, among others, somatic pathogenic NF2 mutations. While the anatomically separate tumors in case 1 were likely molecular identical, the tumors in case 2 showed different genetic patterns. WRS 65 was 55% at 6 years of follow-up and 60% at 4.5 years of follow-up, respectively. CONCLUSIONS: The occurrence of multifocal unilateral cochleovestibular schwannomas without pathogenic variants in NF2 in non-affected blood leucocytes can be associated with mosaic NF2 -related schwannomatosis (case 1), or with likely sporadic mutations (case 2) and may be overlooked due to their extreme rarity. Although challenging, successful hearing rehabilitation could be achieved through surgical resection of the tumors and cochlear implantation.

Sebaceous Carcinoma of the Middle Ear: A Case Report.

Here we present the first case of sebaceous carcinoma of the middle ear. We discuss the treatment course and post treatment results after 11 years of follow up. We further summarize the available literature of sebaceous carcinoma of the temporal bone, which prior to this case was exclusively limited to the external auditory canal. Laryngoscope, 134:3769-3772, 2024.

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.

An Unusual Case of an ALK-Negative Epithelioid Fibrous Histiocytoma in the External Auditory Canal.

This article represents the first reported case in the external auditory canal of epithelioid fibrous histiocytoma (EFH), a rare benign cutaneous epithelioid neoplasm. Immunostaining revealed uncommon negative staining for anaplastic lymphoma kinase (ALK) expression. This case and literature review outline the diagnostic strategy for this highly unusual neoplasm. Laryngoscope, 134:3371-3373, 2024.

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.

A Rare Case of Angiofibroma Presenting as an Endolymphatic Sac Tumor.

A 30-year-old man presented with minute-long episodes of vertigo and severe autophony. CVEMP showed a decreased threshold when testing the left side, potentially indicating SSCD. A subsequent MRI demonstrated a multi-lobulated, cystic mass in the temporal bone and the radiological diagnosis at that time was ELST. Tumor excision was performed, and microscopic examination of the excised material revealed fibrovascular tissue without signs of papillary or cystic projections. The conclusion of the histological assessment rendered a diagnosis of angiofibroma. We were unable to find a previous report of ENA originating around the endolymphatic sac. Laryngoscope, 134:1897-1900, 2024.

Single-stage reconstruction of a large defect of the ear.

To reconstruct a large anterior skin and cartilage defect of the upper half of the external ear in an elderly patient after cancer surgery, different techniques are possible, but single-stage procedures should be advised. Combining flaps with reliable vascular supply, like the revolving door post-auricular flap and a mastoid advancement flap, is an attractive single-stage reconstructive option to rebuild a sturdy auricle.

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.

[Application of supraclavicular fasciocutaneous island flap for reconstruction after removal of tumors in parotid and auricle area].

Objective: To evaluate the efficacy of supraclavicular fasciocutaneous island flap (SIF) for repairing the defect of parotid or auricle regions after tumor resection. Methods: From February 2019 to June 2021, 12 patients (11 males and 1 female, aged 54-77 years old), of whom 4 with parotid adenoid cystic carcinoma and 8 with auricular basal cell carcinoma underwent reconstruction surgery for postoperative defects in the parotid gland area and auricular area with SIF in the Department of Otorhinolaryngology Head and Neck Surgery, the Second Xiangya Hospital of Central South University and their clinical data were retrospectively analyzed. Size of the SIF, time for harvesting SIF, neck lymph node dissection and postoperative complications were recorded. Results: The flap areas were (6-9) cm × (8-13) cm, and the harvesting time for SIF ranged from 40 to 80 min, averaging 51.7 min. The donor sites were directly closed. All patients underwent ipsilateral levels Ⅰ-Ⅲ neck dissection, with 4 cases undergoing additional level Ⅳ neck dissection and 2 cases undergoing level Ⅳ-Ⅴ neck dissection. Of the 12 SIF, 10 were completely survival and 2 had flap arterial crisis with partial flap necrosis, in addition, 1 had donor site wound dehiscence. With follow-up of 10-42 months, there were no tumor recurrences in 10 patients, 1 patient was lost to follow-up at 10 months postoperatively, and 1 patient experienced local tumor recurrence at 11 months after surgery and died 15 months later. Conclusion: SIF is an easily harvested flap with good skin features matching the skin in parotid and auricle regions and less damage to donor site, and this flap has no need for microvascular anastomosis technique. SIF is feasible and effective for repairing defects in parotid and auricle area.

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.