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摘要


周圍神經上皮瘤是一種少見而且受到爭議的腫瘤,直到最近才逐漸確立其地位。本文報告一個在近期診斷而且治療的小兒病例。病人為10歲大的男孩,求診的主訢是在左前臂,上臂及腋窩發現腫塊,其他方面則很正常。作活體切片,光學及電子顯微鏡都顯示腫瘤細胞是神經來源。免疫組識化學研究則可見到染色為神經元特異陸烯醇酶(neuron-specific enolase, NSE)陽性和S-100弱陽性。尿中鄰苯二酚胺代謝物的值在正常範圍內。周圍神經上皮瘤的診斷成立後,我們作了廣泛的切除,並加上放射線和化學治療。病人目前的情況良好。近年來因各種診斷方法的進步,周圍神經上皮瘤的獨立性才為大家接受。我們提出此一病例是希望大家能夠重視,並能有更多的研究結果。

關鍵字

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並列摘要


Peripheral neuroepithelioma, a rare and controversial tumor, has been recently established as an entity. The authors observed its course in a ten-year-old boy in whom the tumor initially presented as enlarging masses over the left axilla, upper arm and forearm. Hisiology, immunohistochemical and ultrastruclural studies showed tumors of neurogenic origin. Urine catecholamine metabolite levels were within normal range. The patient responded well to a combination therapy of wide excision, radiotherapy and chemotherapy. The reports of the past 10 years suggest that peripheral neuroepithelioma is an unique tumor, distinct clinically and pathologically from other ”round” cell malignancies.

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