Bilateral primary carcinoma of the external auditory meatus]

We report an extremely rare case of bilateral primary carcinoma of the external auditory meatus. A 50-year-old man suffered 1 month from left-ear discharge and otalgia. Examination revealed a tumor of the left ear canal and a biopsy showed well-differentiated squamous cell carcinoma. The patient underwent 60 Gy radiotherapy and left subtotal temporal bone resection. A tumor with irregular swelling of the right external canal was found 8 months after the first diagnosis. Biopsy of the right external canal confirmed the same squamous cell carcinoma. Genetic examination that the carcinoma of the right ear was probably not a metastatic from the carcinoma of the left ear.     

Temporal bone carcinoma.

Squamous cell carcinoma is the most common malignancy of the external auditory canal, middle ear, and mastoid. Surgical resection is the cornerstone of treatment, with T1 lesions of the external auditory canal treated by lateral temporal bone resection and more advanced lesions treated by subtotal or total temporal bone resection. This article summarizes clinical manifestations, differential diagnosis, management, and differences in treatment philosophy.     

Adenoid cystic carcinoma of the external auditory canal

Adenoid cystic carcinoma of the external auditory canal is much less common than squamous cell carcinoma and basal cell carcinoma. Signs and symptoms of the case presented here consisted of a longstanding discharging ear, a progressive hearing loss, and the presence of a circumferential subcutaneous induration in the distal third of the external auditory canal. No dizziness, tinnitus, or facial weakness was present. Computed tomography showed a soft tissue mass in the external auditory canal and middle ear with no lytic areas of the mastoid or the temporal region. A biopsy specimen was interpreted as an adenoid cystic carcinoma. Treatment consisted of radical excision of the external auditory canal via a modified temporal bone resection. This radical approach resulted in the local eradication of the lesion.     

Pseudoepitheliomatous Hyperplasia Versus Squamous Cell Carcinoma of the External Auditory Canal

Four case reports are presented to demonstrate the clinical and histopathologic similarity of pseudoepitheliomatous hyperplasia (PH) to squamous cell carcinoma (SCC) in the external auditory canal (EAC). In all four cases the original report of SCC on a biopsy specimen of an EAC lesion was corrected on review to PH. In one patient conservative management resulted in resolution of the EAC lesion. A second patient underwent radiation therapy and partial temporal bone resection with no SCC found in the surgical specimen. A third patient's ear canal had healed with conservative treatment and repeated biopsy revealed no malignancy. After a 6-year symptom-free interval, she developed invasive SCC with bone involvement that required surgery and radiation treatment. A fourth patient underwent a sleeve resection of the skin of the EAC that proved to be PH, and no evidence of SCC was found. A thoughtful clinical history, careful physical examination, response to conservative treatment, and close communication with the pathologist should be exercised in the evaluation of EAC lesions.     

Bilateral squamous cell carcinoma of the external auditory canals

OBJECTIVES: To report a case of bilateral squamous cell carcinoma of the external auditory canals and to present a management algorithm for this difficult clinical scenario. STUDY DESIGN: Case report and literature review. METHODS: The study comprises a case report of a 69-year-old man who initially presented with complaints of bilateral otorrhea, left-sided otalgia, and a left-sided hearing loss. Following attempted treatment of a presumed case of otitis externa, biopsy of both external auditory canals revealed squamous cell carcinoma. A computed tomography scan demonstrated marked abnormal soft tissue in the left external auditory canal with no bony erosion and thickening of the soft tissue in the right external auditory canal. RESULTS: The left-sided lesion required a lateral temporal bone resection, a partial superficial and deep-lobe parotidectomy, and postoperative irradiation. The right-sided lesion was more limited and was managed with a lateral temporal bone resection and tympanoplasty for hearing preservation. CONCLUSIONS: Bilateral squamous cell carcinoma of the external auditory canals is an extremely uncommon but aggressive malignancy that may present with symptoms similar to a case of otitis externa, and this can result in delays in proper diagnosis. Early recognition is essential because management and prognosis are determined by the extent of the lesion.     

颞骨骨化纤维瘤

目的：探讨颞骨骨化纤维瘤的诊断和治疗，提高对该病的认识。方法：报道1例颞骨骨化纤维瘤男性患儿，取耳后沟外切口，暴露瘤体，彻底切除肿瘤组织，封闭外耳道残端，取自体腹壁脂肪填塞术腔；术后定期随诊。结果：术后病理证实为颞骨骨化纤维瘤，术后随访1年无复发，外耳道内端封闭完好。结论：颞骨骨化纤维瘤临床罕见，虽为良性肿瘤，但可压迫和侵袭邻近重要组织和器官，引起功能障碍和颅内感染,一旦确诊，应尽早完整切除。     

Primary pleomorphic adenoma of the external ear canal. Report of a case and literature review

INTRODUCTION: Primary neoplasms of the external ear canal are rare, and 5% of these tumors are of glandular origin. Ceruminal glands are modified sweat glands of the skin of the external auditory meatus that may give rise to (a) benign tumors such as ceruminous adenoma, pleomorphic adenoma, and syringocystadenoma papilliferum, and (b) malignant tumors such as ceruminous adenocarcinoma, adenoid cystic carcinoma, and mucoepidermoid carcinoma. An alternative theory holds that pleomorphic adenomas may well arise from ectopic salivary tissue present in the external ear canal. These tumors are very rare and usually have a benign course. In this report, we describe an unusual case of pleomorphic adenoma of the external auditory canal associated with chronic infection of the middle ear. CASE REPORT: A 60-year-old woman presented with exacerbation of left otalgia over a 6-month period. She had been affected with chronic otitis media and aural polyps for the last 13 years, for which she had received medical treatment only. A canal-filling aural polyp was noted on clinical examination. Subsequent biopsy and histologic examination revealed pleomorphic adenoma of the external ear canal, possibly with malignant elements. Magnetic resonance imaging showed no intracranial extension or any association with the adjacent parotid gland. The patient underwent modified radical mastoidectomy and complete resection of the tumor and the entire skin of the external auditory canal. Final histology and immunohistochemistry confirmed the absence of malignancy, and no recurrence has been reported 1 year postoperatively. CONCLUSIONS: Pleomorphic adenoma is an extremely rare tumor arising from the ceruminal glands of the external ear canal. Nonspecific presentation and difficult histologic diagnosis characterize this benign neoplasm. Wide local excision is the mainstay of treatment.     

Merkel cell tumour of the external ear. Report of a case

Merkel cells carcinoma (MCC) is an uncommon skin lesion, considered a malignancy of the neuroendocrine system, which is found mainly in elderly people. Its incidence is highly correlated with sun exposure or immunodeficiency syndromes. MCC is often an aggressive tumour with high tendency for local recurrence, lymph node involvement and distant metastasis. To our best knowledge 20 cases originated from the auricle have been described, 2 of them arising from external ear canal. The authors report a case of the ear canal characterized by two others synchronous tumours and the occurrence of a malignant high grade lymphoma, in which contribute of the pathologist was essential for a critical review. MCC diagnosis is not always easy for its pathological and clinical features and it should always be considered in presence of lymphoma. A multidisciplinary approach is basic.     

A case of eosinophilic granuloma in the temporal bone

The following case reports a 4-year-old boy with a solitary eosinophilic granuloma in the right temporal bone. The patient complained of an inflammatory tumor in the right external ear canal. The histopathological diagnosis made based on the first specimen of the tumor in the right ear canal was foreign body granuloma. A polyp developed again in the right ear canal with postauricular swelling. Computerized tomography of the skull at that time showed an osteolytic defect in the right temporal bone filled with soft tissue. Diagnosis was established by finding of the presence of Birbeck granules in Langerhans histiocytes by electron microscopy.     

The adenoid cystic carcinoma

The adenoid cystic carcinoma arising in the external auditory canal is a rare tumor, as other malignant neoplasias of the EAC and middle ear. The early diagnosis of these malignant tumors of the ear is very important to improve the prognosis. In this sense, the clinic experience is frequently the most useful diagnosis instrument to suspect this pathology when we have a confrontation with chronic otorrea and otalgia, common presentation form of this disease. In this report we show a new case of adenoid cystic carcinoma of external auditory canal and review the most significant medical literature existing in relationship with this pathology.     

Hyper immunoglobulin-E syndrome: a case with chronic ear draining mimicking polypoid otitis media

The hyper-IgE syndrome is a rare, complex immunoregulatory disorder characterized by pruritic dermatitis, recurrent staphylococcus skin abscesses and extremely elevated levels of IgE in serum. In this report, an 8-year-old girl with hyper-IgE syndrome is presented. She had pruritic dermatitis and skin abscesses on her extremities and scalp. Discharge and granulation tissue in right external ear canal were recognized in otorhinolaryngologic examination. Cultures of the suppuration of the external ear canal yielded S. aureus. Biopsy of the lesion was reported as granulation tissue. Local treatment of the ear canal was performed with ear wick soaked with steroid and antibiotic solutions two times per day.     

MMP9蛋白与微血管密度在外耳道及中耳鳞状细胞癌中的表达及其临床意义

目的:检测MMP9蛋白与微血管密度(MVD)在外耳道及中耳鳞状细胞癌中的表达,并研究其临床意义.方法:采用免疫组织化学SP法检测26例外耳道及中耳鳞状细胞癌组织中MMP9与CD34标记的MVD.结果:外耳道及中耳鳞状细胞癌组织中MMP9表达阳性率为73.1%(19/26),正常外耳道皮肤中阳性表达率为25.0%(5/20),2组间比较差异有统计学意义(x<'2>=12.615,P<0.05);外耳道及中耳鳞状细胞癌组织中CD34标记的MVD表达为33.58±3.04,正常外耳道皮肤中表达为22.50±5.22,2组比较差异有统计学意义(P<0.01).外耳道及中耳鳞状细胞癌组织中MMP9蛋白的表达和MVD与组织学分级和肿瘤分期密切相关,而在不同年龄和性别的患者中其表达差异无统计学意义.MMP9和CD34蛋白的表达经Pearson相关检验呈正相关关系(r=0.42,P<0.05).结论:MMP9可能参与了外耳道及中耳鳞状细胞癌的发生,在外耳道及中耳鳞状细胞癌的侵袭和转移中发挥着重要作用,联合检测MMP9与MVD可望成为外耳道及中耳鳞状细胞癌早期诊断和预后判断的分子指标之一.     

Syringoid eccrine carcinoma of external auditory canal. A case report

First described in 1969, syringoid eccrine carcinoma (SEC) is a rare cutaneous tumor with some controversy regarding its correct definition. It consists of solid nests and small cords in a dense fibrocollagenous stroma. As it is rare, its clinical appearance is not well characterized and its biological behaviour is not defined. It usually affects skin of the scalp, extremities and more rarely, other sites. It behaves as locally aggressive tumor but metastases are rare. Although there have been some previous reports describing clinical presentation and management of SEC in the skin, there has been no previous reports describing clinical findings and management of this tumor in the external auditory canal. We report a case of a 57-year-old female with small solitary mass in left external auditory canal associated with discharge, severe itching and bleeding on manipulation. Complete local excision is the recommended method for diagnosis and treatment of this tumor in the external auditory canal. This extremely rare case serves as a springboard for the diagnosis as well as the management of SEC in external auditory canal.     

Plasmacytoma of the mastoid bone: solitary and systemic

Plasma cell tumours of the mastoid are rare malignancies of haematological origin. Two patients are described with a plasma cell tumour in the mastoid bone. In one patient it concerned a solitary plasmacytoma of the bone and in the other a focus in the mastoid of a recently diagnosed multiple myeloma. Symptoms in these patients were non-specific. Computed tomography (CT) and magnetic resonance image (MRI) scanning showed a non-specific space-occupying lesion in the mastoid. The diagnosis was made on immunohistological examination, that showed diffuse sheets of monoclonal plasma cells in a clear matrix. When a plasmacytoma lesion is found multiple myeloma should always be excluded. Treatment in case of solitary plasmacytoma of the mastoid bone consists of radiotherapy. In case of localization in the mastoid of multiple myeloma the treatment consists of palliative chemotherapy.     

外耳道胆脂瘤并发化脓性腮腺炎1例并文献复习

目的 探讨外耳道胆脂瘤并发化脓性腮腺炎病因及诊治经验,并进行外耳道胆脂瘤诊疗相关文献复习。 方法 回顾性分析1例外耳道胆脂瘤并发化脓性腮腺炎病例资料,主要症状为左耳听力下降伴流脓,左侧面部红肿疼痛。颞骨CT及耳部核磁示:左侧外耳道内胆脂瘤形成,累及乳突、鼓室、鼓窦、腮腺及咽旁间隙。临床诊断:外耳道胆脂瘤(左,Holt Ⅲ期)、化脓性腮腺炎(左)。手术方式为左耳外耳道胆脂瘤切除术、开放式乳突根治术、鼓室成形术、人工听骨植入术、耳甲腔成形术、腮腺脓肿清除术及腮腺瘘修补术。 结果 术中彻底清除外耳道及中耳内胆脂瘤及腮腺脓肿,并修复腮腺瘘。术后随访患者恢复良好,无胆脂瘤复发残留及腮腺炎复发相关症状出现。 结论 外耳道胆脂瘤具有骨质破坏的潜能。而HoltⅢ期外耳道胆脂瘤并发化脓性腮腺炎病例罕见,明确病因并依据病变侵袭范围选择个体化的治疗方案尤为关键。     

Temporal bone central giant-cell granuloma presenting as a serous otitis media

Central giant cell granuloma is a benign intraosseous lesion that most commonly occurs in the facial bones. Its location in the temporal bone is extremely rare and only 20 cases have been reported in the literature. We report a case of an adult female patient presenting with a right serous otitis media and mastoiditis associated with a mixed hearing loss during 6 months. CT-scan and MRI revealed a temporal bone tumor involving the mastoid, and surrounding the right temporo-mandibular joint. Tumor was totally removed after a canal-wall-down mastoidectomy and middle ear exclusion. Pathology revealed a central giant cell granuloma. Seven months following the surgery there was no evidence of recurrence. Central giant cell granuloma is a rare temporal bone lesion, with non specific clinical and imaging signs but characteristic pathological features. Today, a total surgical removal and regular MRI follow-up is the best management option.     

中耳间变性大T细胞淋巴瘤1例并文献复习

目的 探讨中耳间变性大T细胞淋巴瘤的临床特点及诊疗方法,以便对其进行早期诊断.方法 回顾性分析1例中耳间变性大T细胞淋巴瘤患者的临床资料并复习相关文献进行总结.结果 淋巴瘤多发生于骨骼、骨髓、皮下组织和脾脏,发生于中耳者罕见.中耳淋巴瘤起病时均无浅表淋巴结肿大,通常表现为耳痛、听力下降及耳内溢液(浆液性、脓性或血性),...     

Primary malignant melanoma of the external auditory canal: a case report with presentation as an aural polyp.

A case of primary cutaneous melanoma of the external auditory canal and a review of the literature are reported. This is the first reported case with microstaging of the tumor. The tumor presented as a polypoid obstructive lesion of the external ear canal. Although occurrence of melanoma in this location is extremely rare, differential diagnosis of an external ear canal polyp should be expanded to include primary malignant melanoma.     

Malignancies of the external ear canal and temporal bone: surgical techniques and results

A combined therapy approach to malignancies of the external auditory canal and middle ear has been developed. A technique of external canal resection and gross tumor removal from the middle ear, parotid gland, and superior cervical lymph nodes is followed by postoperative full-therapy irradiation. This combined approach has been used in 30 patients with malignancies involving the external auditory canal and temporal bone. The preoperative evaluation and surgical technique, including the intraoperative decision-making process, is described. Twenty-four patients had squamous cell carcinoma of the external auditory canal, and two patients had basal cell carcinoma. There was one patient each with adenocystic carcinoma, acinic cell carcinoma, high grade mucoepidermoid carcinoma, and a giant cell tumor of bone. This group of patients was broken down into three groups based on the extent of disease as determined at surgery. Overall control of disease, both locally and distant, for the 30 patients was 66%. There were 12 patients with disease limited to the ear canal. These patients had a 91% survival of this disease process. Seven patients were determined to have limited extension beyond the ear canal. These were treated with combined therapy with an overall control of disease of 72%. Eleven patients had extensive disease outside of middle ear into the carotid jugular spine, stylomastoid foramen, and skull base, with a survival rate of 45%. It is concluded that a step-wise removal of all gross tumor, as opposed to an en bloc dissection of the temporal bone and skull base, followed by full-therapy irradiation gives equally as good, or even better, long-term survival for this malignancy of the external auditory canal and middle ear.     

Two cases of myxoma of the external auditory canal

Myxomas were found in the outer ear canal of a 66-year-old female and a 51-year-old male. Both tumors were soft and sessile and located at the antero-superior part of the external auditory canal near the eardrum. Profuse, clear mucus was seen when the tumor ruptured or was incised. Histology showed spindle-shaped and stellate cells in myxoid extracellular matrix. CT images revealed a small bone defect adjacent to the tumor in the meatal bone. This bony defect was confirmed during surgery. The bony defects seen in both cases may have something to do with the genesis of the tumor. We found only 5 sporadic cases, including ours, reported in the English literature. As myxoma of the outer ear canal is also reported in 18 patients as a symptom of Carney's complex, we must check for accompanying symptoms of the complex such as spotty skin pigmentation, endocrine tumors, or schwannoma. Total removal of the tumor is necessary to prevent recurrence.