- 期刊
Neuroendocrine Adenoma of the Middle Ear-A Case Report and Literature Review
中耳神經內分泌腺瘤－案例報告及文獻回顧
摘要
Neuroendocrine adenoma of the middle ear (NAME) is a rare neoplasm of the middle ear, comprising less than 2% of all middle ear tumors. The clinical presentation of NAME is often nonspecific and can include unilateral hearing loss, aural fullness, tinnitus, vertigo, and facial palsy. Diagnosis of NAME can be challenging, as a pre-operative clinical diagnosis is difficult to make. Definitive diagnosis is based on histopathology and immunohistochemistry (IHC) findings. In this case report, we present the case of a 41-year-old woman who presented with a one-year history of unilateral hearing loss and tinnitus on the right side. Examination with an otoscope revealed a pale-reddish mass in the right external auditory canal. Pure tone audiometry showed right conductive hearing loss with a 12.5 dB air-bone gap. Computed tomography revealed a soft tissue lesion in the right external auditory canal, middle ear, attic space, and aditus ad antrum, encasing of ossicles. Magnetic resonance image displayed a tumor with isointense to hyperintense signal in T1 and T2-weighted image with contrast enhancement. The initial diagnostic impression was paraganglioma, and surgical intervention was deemed necessary. The tumor was excised through a post-auricular approach with the assistance of a microscope and endoscope, revealing that the tumor was occupying the middle ear, attic space, and aditus ad antrum. A rigid endoscope was used to confirm the absence of residual tumor. The result from an intraoperative frozen section favored a paraganglioma. However, a postoperative histological and immunohistological examination confirmed the tumor as a NAME. The post-operative air-bone gap decreased to 5 dB. The patient underwent regular follow-up for 2 years, during which no evidence of recurrent disease was observed. We conclude that endoscope-assisted microscopic surgery is effective in reducing residual lesions and achieving complete tumor resection while preserving the ossicles. Given that NAME is considered a low-grade malignancy, long-term follow-up is necessary.
並列摘要
中耳神經內分泌腺瘤（Neuroendocrine adenoma of middle ear）為罕見的中耳腫瘤，約僅佔所有中耳腫瘤的2%。其臨床表現不具特異性：如單側聽力下降、耳塞感、耳鳴、暈眩甚至顏面神經麻痺。術前較難與其他中耳腫瘤區別，多由切片或手術後病理診斷。在此報告一個中耳神經內分泌腺瘤案例，病人是41歲女性，因右側聽力下降合併耳鳴的情形約1年，理學檢查發現右側外耳道後上壁腫塊。純音聽力檢查顯示左側傳導性聽損，氣骨導差12.5dB。顳骨細切電腦斷層顯示位於中耳腔延伸至外耳道、且包埋住聽小骨的軟組織腫塊。核磁共振顯示為T1、T2影像皆為中至高密度信號、有顯影之腫瘤，起初懷疑為鼓室血管球瘤，因此安排手術切除。手術採耳後路徑、合併顯微鏡及內視鏡手術，術中發現腫瘤包埋且侵蝕砧骨，完整將中耳及外耳道腫瘤移除後，使用內視鏡仔細確認中耳內無殘餘腫瘤。術中冷凍切片結果顯示為副神經節瘤，最後病理報告結果則為中耳神經內分泌腺瘤。術後氣骨導差下降至5dB。目前追蹤兩年，無復發跡象。我們認為使用顯微鏡合併內視鏡手術能夠在保留聽小骨的狀況下，確保腫瘤完整移除，降低未來復發的機率，因為中耳神經內分泌腺瘤為低惡性度腫瘤，因此病人需要接受長期規則追蹤。