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摘要


淋巴管平滑肌瘤症是一種只發生在生育年齡女性的進展性疾病。它是一種非常罕見的疾病,但在臨床、放射線診斷學及病理學上都有其特異的表現。 我們報告一個經手術証明為淋巴管平滑瘤症的病例。病人是29歲女性,臨床表現為再發性氣胸。胸部X光表現為兩側網狀及蜂巢形態,HRCT(高解像電腦斷層掃描)表現特徵是瀰漫性囊狀變化。病理表現為平滑肌細胞增生併隨淋巴管擴大。了解此病在放射線學上的表現,對其術前診斷有相當幫助。

並列摘要


Lymphangioleiomyomatosis (LAM) is a progressive disease occuring exclusively in women of child-bearing age. It is a rare disease, possessing distinctive clinical, pathological and radiological findings. A case is reported of surgically- proved lymphangioleiomyomatosis in a 29-year-old woman with recurrent pneumothorax. Chest plain film showed bilateral reticular and honeycomb patterns with a normal lung volume. High resolution computed tomography (HRCT) revealed characteristic diffuse cystic changes of the lungs. Recognition of this characteristic radiological feature is helpful in suggesting the diagnosis of LAM.

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