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摘要


惡性肌上皮瘤(Malignant Myoepithelioma)為一罕見之唾液腺腫瘤,迄今文獻上報告過之病例少於100例,發生部位以腮腺最常見、其次為頷下腺及軟硬勝,發病年齡平均為55歲,無性別上之差異;症狀呈現為無痛硬塊且持續數月或數年直至診斷確認。組織學診斷時使用一般光學顯微鏡並不容易檢查出,需施以特殊免疫染色檢查才可確立診斷。依據文獻治療方式仍以手術切除為主,至於放射線及化學治療的療效則尚未被證實。文獻報告惡性肌上皮瘤亦有局部復發及頭部與遠端轉移等情形發生,因此長期術後追蹤有其必要性。本報告提出一追蹤長達8年之病例。病例首次在1998年因右上腭黏膜腫塊接受切除手術,術後病理組織報告為右上腭黏膜肌上皮瘤(Myoepithelioma)。追蹤至第四年出現同側頸部轉移,回溯組織病理重新確認診斷1998年之病變為右上腭黏膜急性肌上皮瘤;此後病患於第七年出現局部復發之現象,並接受切除手術;追蹤至今與任何復發及轉移出現。本研究將針對此病例之診斷與治療詳細紀錄,並回顧惡性肌上皮瘤之相關文獻進行討論。

並列摘要


Malignant myoepithelioma is an extremely rare tumor of the salivary glands, and less than 100 cases had been reported. Most of malignant myoepithelioma located in the parotid gland, then submandibular and minor salivary glands. The mean age at presentation is 55 years old, no gender distribution difference. Clinical features were painless, slow growing firm mass, usually takes months or years before define the disease. Histological characters revealed wide variation makes the diagnosis with light microscope examination uneasy. Immunohistochemical staining with specific tumor markers to confirm the final diagnosis is necessary in most cases. Surgical eradication of the tumor is the first choice of treatment modality, no significant response to radiotherapy or chemotherapy. The distant metastasis potential and high local recurrence rate had been reported. In this article, we revealed a malignant myoepithelioma of right palatal mucosa case, both right cervical metastasis and local recurrence experienced, but still survived with disease free until now for over 8 years. During the treatment period, different clinical and histological features were reviewed, monitoring treatment modality outcome and carefully follow up were also recorded. Report the case and reviewed malignant myoepithelioma literature were also done.

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