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成人鼻腔橫紋肌肉瘤-病例報告

Rhabdomyosarcoma of the Nose in an Adult - Case Report

摘要


頭頸部的橫紋肌肉瘤在臨床上並不多見,一般多發生於小孩,少見於大人。橫紋肌肉瘤在頭頸部好發於眼窩和腦膜附近(包括中耳、鼻咽、副鼻竇和鼻)。組織學上大多為胚胎型橫紋肌肉瘤,而多形性型則較少見。一般預後差,但近15年來由於除了手術和放射治療外,加入了綜合性化學治療,使得存活率小於20%進步到70%左右。馬偕醫院於民國80年3月經歷一例成人鼻腔多形性橫紋肌肉瘤,經手術完全切除後並給予放射和綜合性化學治療。今將其臨床表徵,病理學,及近年來治療趨勢作一文獻上的回顧。

並列摘要


Rhabdomyosarcoma in the head and neck is an uncommon tumor found mainly in children. The usual sites of occurrence in the head and neck are the orbit and the parameningeal sites, which include the middle ear, nasopharynx, paranasal sinuses and the nose. The embryonal type is the most common rhabdomyosarcoma of the head and neck region and is usually found in children. The pleomorphic type is rarely seen and usually found in the adult. The overall prognosis is poor, but due to the recent addition of multimodal chemotherapy to the treatment of this tumor, the 5 year survival rate has improved from less than 20% to around 70%. We present a case of pleomorphic rhabdomyosarcoma of the nasal cavity in an adult patient treated by combination of surgery, postoperative radiotherapy and chemotherapy. The clinical and histological findings and recent trends in the treatment of this tumor are presented and discussed.

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