PULMONARY LYMEHANGIOMYOMATOSIS- A CASE REPORT

肺部淋巴管平滑肌瘤--病例報告

紀慶鍠(Ching-Hwang) ； 朱海榮(Jih Chu-Hoi Weng) ； 李覃(Tain Lee) ； 何霖(Willian L. H) ； 楊建芳(Chien- Fang Yang)

《中華放射線醫學雜誌》 13卷2期 (1988/06) Pp. 217-220

摘要

Lymphangiomyomatosis is a rare but extremely interesting condition caused by progressive widespread smooth muscle proliferation in the perilymphatic regions throughout the lungs. To date, lesser than 100 cases have been described in the literature. The sex incidence shows an overwhelming female predominance. The most of the patients in the 30 to 50 years of age. Dyspnea is the commonest symptom. Characteristic chest roentgenographic findings are: diffuse interstitial lung disease pleural effusion, recurrent pneumothorax, increase lung volume. Although rare I these cases can be readily recognized by the combination of a very characteristic clinical picture and a typical roentgen pattern.

關鍵字

lymphangiomyomatosis

並列摘要

肺部淋巴管平滑肌瘤是一種罕見病例，其主要由於肺部內淋巴管周圍的平滑肌，廣泛且漸進式的增生所造成，至目前為止，在文獻上少於一百個病例被提出來報告。肺部淋巴管平滑肌瘤好發於女性；大多數在30歲至50歲之間病人，在臨床上，呼吸困難為最常見的症狀；胸部X光主要特徵為肺部瀰漫性間質的病變，肋膜積水，再發氣胸，肺容積增加。這些病例往往可由臨床資料及典型X光特徵而獲得診斷。