Case report: rare presentation of primary angiomyolipoma in the middle ear
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CASE REPORT
Case report: rare presentation of primary angiomyolipoma in the middle ear Fei Wang1 and Hao Wang2*
Abstract Background: Angiomyolipoma (AML) is a common, chronic disease usually localized in kidney and liver organs; but occurring frequently outside the kidney or liver. Case presentation: We describe an unusual case of a 62-year-old female patient with AML in the middle ear. She presented with left earache, tinnitus and hearing loss. Preoperative computed tomography and magnetic resonance imaging seemed to reveal a middle ear cholesteatoma. The patient underwent surgical resection of this lesion, and the AML was finally confirmed by histopathological examination. The patient was discharged on the 8th postoperative day and did not seek further treatment. Conclusions: Extraperitoneal AML is rare and accurately identified by histopathology. The recommended management is surgery for AML in the middle ear. Keywords: Middle ear angiomyolipoma, Diagnosis, Treatment Background Angiomyolipoma (AML) is the most common benign tumour of the kidney and is related to the tuberous sclerosis complex, which is composed of vascular endothelial cells, smooth muscle cells and fat cells [1–3]. Patients with tuberous sclerosis complexes often have multiple renal AMLs [4]. Additionally, AML is frequently found among women with lymphangioleiomyomatosis [5]. Both incidental and disease-related AML are caused by mutations in the TSC1 or TSC2 genes that control cell growth and proliferation [6]. However, this tumour outside the kidney is extremely rare. Given the paucity of information about this tumour, the aim of this study was to present a case, the first AML reported in the middle ear, to describe the clinical features and a treatment protocol.
*Correspondence: [email protected] 2 Department of Intensive Care Unit, People’s Hospital of Qinghai Province, Xining, China Full list of author information is available at the end of the article
Case presentation A 62-year-old female patient was admitted to the hospital in July 2019 because of left earache, tinnitus, hearing loss for 30 years and worsening earache for more than 1 month. She had not received any treatment before. The patient’s personal history, past history and family history were unremarkable. Renal ultrasound, lung computed tomography (CT) and skin examination were performed in this patient to preclude the potential AML of other parts of the body outside the middle ear. On physical examination, the bilateral auricles showed no deformity or traction pain, and a large amount of purulent secretions were found in the left external auditory canal. Cerumen debris was found in the right external auditory canal, and no obvious tenderness was found in the bilateral mastoid process area. The rinne test results were as follows: left (+), right (−). The weber test result was right-sided (+). Temporal bone CT showed right otitis media mastoiditis, partial defect of the left temporal bone, no normal structure in the middle ear cavity and soft tissue thi
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