4 Lungs, kidneys, and vertebrae are the most commonly involved sites. ![]() 1,3,4 Distal metastasis are more frequent and occur in 48% of the patients, mainly when the primary tumor was not completely removed. Neck clearance is offered only if positive nodules are found in the neck, which happens approximately in 4% of the cases. Some authors recommend radiotherapy only in advanced tumors when there is skull base invasion, neck metastasis, perineural invasion, solid histological type, and relapsing tumors. 2,5,6 Post-operative radiotherapy is also recommended to help control local tumor recurrence (86% against 11% when only surgery is done) 1,3,4. Radical mastoidectomy coupled with ear canal resection and specimen freezing to assess margins and perineural invasion is the treatment of choice. Fine needle aspiration has been used to assist in the preoperative diagnosis. 5,6ĭiagnosis is done through pathology examination. 1 Symptoms are pain, hypoacusis, otorrhea and EEC nodule, and the tumor usually grows for years before the patient is diagnosed. 1,5 The tumor may appear at any age, but incidence peaks in the 5th and 6th decades of life. have seen increased incidence among males. found it to be more frequently found in women, while Lucia A et al. There is controversy in the literature as to gender prevalence. The characteristics are the same as encountered in small salivary gland ACC, 5,6 with silent growth, local recurrence, perineural invasion, and late distal metastasis. However, among the glandular tumors, adenoid cystic carcinomas are the most prevalent. The patient was referred to additional treatment with radiotherapy four weeks after surgery.ĮEC tumors are rare and only 20% have glandular origins. This time the pathology tests showed no presence of disease. ![]() A modified radical mastoidectomy was then performed and the entire ear meatus was removed, including the region of the tragus. Intracanal resection surgery was performed and pathology tests classified the tumor as a cribriform adenoid cystic carcinoma with compromised margins. The tumor was located in the superior-anterior portion of the left EEC. The borders were partially defined, and the neoplasm measured 1.46 cm in its widest point. 1) and a solid, round, average density lump enhanced by contrast was found. CT scans of the temporal bones were ordered ( Fig. During physical examination a tumor of fibroelastic consistence was found in the upper wall of the external ear canal, occupying nearly the entire diameter of the meatus. 5,6 CLINICAL CASEĪ 77-year-old female patient came to our service complaining of intense left otalgia present for the past 6 months, accompanied by mild hypoacusis and a slow-growth lump in the left outer ear meatus. It is characterized by perineural invasion and distal metastasis, as in other adenoid cystic tumors. Symptoms are pain, hypoacusis, and slow-growth nodule. Tumors in the EEC are quite rare, as glands are the preferential site for adenoid cystic carcinomas. 1–4 It is found more often in the smaller salivary glands 1–3, but may also appear in lacrimal glands, tracheobronchial tree, breasts, esophagus, and external ear canal (EEC). Adenoid cystic carcinoma is a rare disease, as it accounts for 3–10% of the salivary gland tumors and 1–4% of all head and neck neoplasms.
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