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.

This report presents a rare case of bronchogenic adenocarcinoma with initial metastasis in the external auditory canal. The patient, a 64-year-old man with a history of bladder urothelial carcinoma, initially presented with a persistent right otitis externa. Otoscopic examination revealed a mass obstructing the right external auditory canal. The temporal bone computed tomography scan revealed a mass that completely obstructed the right external auditory canal and extended into the middle ear. A biopsy showed a poorly differentiated adenocarcinoma of bronchogenic origin, confirmed by positive immunohistochemical staining for cytokeratin 7 and Thyroid transcription factor-1. Further imaging revealed a large tumor mass in the lung involving the mediastinum and parenchyma, along with carcinomatous lymphangitis and cerebral metastasis. Histopathological examination of the primary lung tumor confirmed a poorly differentiated adenocarcinoma with similar features to the metastasis in the external auditory canal. The tumor was staged as T4N2M1c, and the patient underwent local external-beam radiation therapy with chemotherapy.

Trichoblastoma（TB） is a rare germ cell skin adnexal tumor of the hair, and it is a rare follicular tumor of the skin that differentiates from the hair germ epithelium and is often regarded as a benign skin tumorHowever, it is poorly confined and has a local infiltrative growth pattern. tb occurs in the head and neck region, especially in the face, and presents clinically as a slow growing, well-defined and elevated nodule. TB is routinely treated surgically. Due to the lack of universally accepted treatment guidelines or protocols, the recurrence rate after surgery is high, which makes clinical cure more difficult. In this study, a 65-year-old female patient was found to have a swelling with recurrent rupture and pus flow from the right external auditory canal opening and the auricular cavity. After initial misdiagnosis as otitis externa, she was treated with conventional anti-infective therapy, but her symptoms did not resolve and gradually worsened before coming to our hospital. The condition presented in this case is relativelyrare,therepre,timely and accurate diagnosis and treatment are crucial for prognosis improvement of such diseases.
摘要: 毛母细胞瘤（trichoblastoma，TB）是一种罕见的毛发生殖细胞皮肤附件肿瘤，罕见的皮肤滤泡肿瘤，分化于毛胚上皮，常被视为良性皮肤肿瘤，但局限性差且具有局部浸润性生长模式。TB好发于头部和颈部区域，尤其是面部，临床表现为生长缓慢、边界清晰及隆起的结节。TB常规以手术治疗为主，由于目前缺乏普遍接受的治疗指南或方案，术后复发率高，为临床治愈增加了一定的难度。本研究报道1例65岁女性患者，发现右侧外耳道口及耳甲腔肿物并反复破溃流脓，最初误诊为外耳道炎，以常规抗感染治疗，症状未缓解并逐渐加重后来我院就诊。该病例所展现的病症极为罕见，因此，及时且准确的诊断与治疗对此类疫病的诊疗和预后至关重要。.

External Auditory Canal Cholesteatomas (EACC), is an exceptionally rare condition with a prevalence of only 0.1-0.5% among new patients1. EACC are known to possess bone eroding properties, causing a variety of complications, similar to the better-known attic cholesteatomas. We describe here the novel surgical management of a case of EACC. She is 38-year-old female who presented with otorrhea for 6 months. Clinical examination and radiological investigations suggested the diagnosis of an external auditory canal cholesteatoma. The patient underwent modified radical mastoidectomy with type 1 tympanoplasty with meatoplasty. Post-operatively, the patient showed marked clinical improvement.

BACKGROUND: Medication-related ear canal osteonecrosis (MRECO) is a growing concern linked to prolonged anti-resorptive medication use. Despite primary care providers being key prescribers of these medications, there is limited information about MRECO in primary care literature. This article presents a case of bisphosphonate-induced osteonecrosis of the external auditory canal (EAC), emphasizing the vital role of primary care providers in identifying this rare yet significant side effect of anti-resorptive medication.
UNASSIGNED: A 65-year-old female, on long-term alendronic acid for osteoporosis, presented to primary care with a 2-year history of left-sided ear blockage and itchiness. Despite prolonged topical treatment for ear wax, symptoms persisted, leading to an Otolaryngology referral. Microsuction revealed exposed bone in the left EAC.
METHODS: A computed tomography scan confirmed bony erosion of the left EAC, and in the absence of other osteonecrosis risk factors, bisphosphonate-induced osteonecrosis was diagnosed. Management involved bisphosphonate discontinuation, regular aural toilet, and topical treatment, achieving complete ear canal epithelialisation within 6 months.
CONCLUSIONS: MRECO, a rare complication of anti-resorptive therapy, is anticipated to rise with increasing antiresorptive medication use in the ageing population. Unexplained ear symptoms in those with a history of current or prior anti-resorptive therapy should raise clinical concern, prompting evaluation for exposed bone in the EAC. Raising awareness of MRECO among primary care providers is crucial for early diagnosis and timely management.

A 48-year-old man presented with a chief complaint of intermittent right ear otorrhea of several-month duration, occasional otalgia and progressive unilateral hearing impairment. He also reported frequent episodes of headache and pressure in the sinuses and maxilla. Previous systemic treatment with antibiotics failed to alleviate the symptoms. A head/neck CT showed completely normal mastoid, middle ear and external auditory canal regions without any evidence of opacification or bone erosion. Otoscopic examination of the right ear disclosed aggregates of dried, brown, fibrillar material and debris occluding the external auditory canal and obstructing the otherwise intact tympanic membrane. Dilation of the external auditory canal or thickening of the tympanic membrane were not appreciated. The canal was debrided and the fibrillar material was placed in formalin. Histopathologic examination revealed numerous branching, septated fungal hyphae organized in densely-packed clusters. In other areas, the fungal hyphae abutted or were attached to lamellated collections of orthokeratin. As highlighted by GMS staining, the fungi were morphologically compatible with Aspergillus species. The clinicopathologic findings supported a diagnosis of fungal otitis externa, while the numerous anucleate squamous cells were compatible with colonization of an underlying, probably developing, cholesteatoma. Culture of material isolated from the external auditory canal confirmed the presence of Aspergillus flavus. In this illustrative case, we present the main clinical and microscopic characteristics of Aspergillus-related otomycosis developing in the setting of a tautochronous cholesteatoma.

BACKGROUND: A malignant myoepithelioma is a rare tumor, mostly arising from the salivary glands. Myoepitheliomas of the ear have rarely been reported. The manuscript reports myoepithelial carcinoma of the external auditory canal (EAC) spreading to the infratemporal fossa. A clinician must be aware of anatomical variation of the bony EAC wall, such as the foramen of Huschke. This rare defect may be a pathway for spreading pathologies between these two anatomical regions.
METHODS: We present a case of osteoma-like stenosis of the EAC, which turned out to be an extremely rare malignant tumor. The preoperative MRI and PET/CT revealed that two parts of the tumor communicated through a defect in the antero-inferior portion of the bony ear canal. No distant metastases were detected. Subsequently, the tumor was resected from the ear canal and the infratemporal fossa en bloc. Perioperatively the defect in the EAC wall was suspected of the foramen of Huschke. After the surgery, the older scans of the patient from the past showed no presence of a congenital EAC wall defect. Therefore, the authors concluded that the tumor aggressively grew through the bone due to its biological nature.
CONCLUSIONS: Malignant myoepithelioma of the external auditory canal is an extremely rare condition and could be misdiagnosed as other benign lesions. In cases of suspicious lesions, it is advisable to do a probatory biopsy from the EAC. Surgery is the treatment of choice in malignant myoepitheliomas, and regular follow-ups are essential to monitor for recurrence or metastatic disease. Any mass located at the antero-inferior portion of the EAC wall warrants close evaluation due to its potential for expansion from the EAC.

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.

Meningiomas are tumours typically derived from the meningothelial cells of the arachnoid mater. They most often arise in intracranial, intraspinal, or orbital locations. Ectopic meningiomas, described as primary meningiomas with no intracranial involvement, are definitely unconventional. In fact, most of the extracranial meningiomas described in the literature, particularly in the outer ear, are effectively spreads of disease with primary intracranial localization. We describe a case of a primary external auditory canal meningioma with demonstrated absence of intracranial involvement, and we provide a full radiological, histological, immunohistochemical and molecular characterization of the lesion.

Mucoepidermoid carcinoma is a malignant neoplasm of exocrine glands that arises predominantly in salivary glands. It is seldom encountered as a primary cutaneous neoplasm, and in those patients, it often involves the external auditory canal. Given their rarity, they can pose a diagnostic challenge and prompt extensive workup. In salivary glands, mucoepidermoid carcinomas commonly harbor CRTC1/3::MAML2 fusions; however, genetic alterations of primary cutaneous neoplasms are less characterized, with previous studies reporting CRTC1 rearrangements in the absence of MAML2 aberrations. Herein, we report a case of a primary cutaneous mucoepidermoid carcinoma of the external auditory canal with a CRTC1::MAML2 rearrangement. We also review the clinical, morphologic, and molecular features of this neoplasm and compare them to those reported in the literature and to histopathologic mimics.