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題 名 | Pulmonary Lymphangioleiomyomatosis: A Clinicopathological Analysis of Ten Cases=肺臟多發性淋巴平滑肌瘤:10例之臨床病理分析 |
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作 者 | 翁世樺; 劉會平; 吳怡成; 蔡熒煌; 林鴻銓; 林孟志; 林坤榮; 黃秀芬; | 書刊名 | 長庚醫學 |
卷 期 | 27:3 2004.03[民93.03] |
頁 次 | 頁201-209 |
分類號 | 415.468 |
關鍵詞 | 肺臟多發性淋巴平滑肌瘤; 高解析度電腦斷層攝影; 荷爾蒙療法; Lymphangioleiomyomatosis; High-resolution computed tomographic scan; HMB-45; Hormone therapy; |
語 文 | 英文(English) |
中文摘要 | 背景:肺臟多發性淋巴平滑肌瘤是一個非常罕見之疾病,多好發於有生育能力時期之女性。目前在臺灣這一類病例的報告仍十分少見。 方法:我們從病理報告檔案以及肺臟多發性淋巴平滑肌瘤之病例。這些確認之病例必須有確切病理切片之診斷和典型高解析度電腦斷層攝影之表現。臨床表現及追蹤之資料皆依據病歷之記載。所有仍找得到石蠟塊組之病例,我們皆加做HMB-45,動情激素受體(ER),和黃體素受體(PR)之免疫染色。 結果:從1990年到2001年,我們總共找到10個有完整臨床及病理資料之病候全部皆為女性,追蹤期間由18個月到167個月不等。有6個病人曾接受爾蒙療法。依據理學檢查及其肺功能檢查結果,6個病人在追蹤期間皆很穩定,4個病人其臨床病狀則有逐漸惡化的現象。有兩個病例因接受肺臟移植手術及後腹腔手術死於另外一定醫院。 結論:我們這一系列病人之臨床及病理表現皆和其他國定之報告相似。所有10例病患,其臨床症狀之進展皆十分緩慢。荷爾蒙療法無明顯療效。病理切片為重要診斷依據,但切片之大小及位置也會影響其準確性。 |
英文摘要 | Background: Lymphagioleiomyomatosis (LAM) of the lung is a very rare disease of unknown etiology which occurs particularly in women of childbearing age. So far, there have been very few reports of LAM in Taiwan. Methods: Data on all patients with a clinical or pathological diagnosis of LAM in Chang Gung Memorial Hospital from 1990 to 2001 were collected from the pathology files or by consultation with thoracic surgeons, chest physicians, and radiologists of this institution. Cases were confirmed by lung biopsy and high-resolution computed tomographic scanning. Clinical data were obtained form patients’ charts. Additional immunostaining for HMB-45 antigen, estrogen receptor, and progesterone receptor was carried out for cases with available paraffin blocks. Results: In total, 10 patients confirmed with a diagnosis of LAM and adequate clinical follow-up information were found from 1991 to 2001. all were females of fertility age. The follow-up periods ranged from 18 to 167 months. Six patients received hormonal therapy. According to the pulmonary function tests and clinical symptoms, 6 patients were in stable condition and 4 showed slow progression of disease by the time of the last follow-up. Two patients died after lung transplantation and abdominal surgery for retroperitoneal LAM, respectively, at another medical center. Conclusions: The clinciopathological features of our series are similar to those reported from other countries. The clinical course was slowly progressive with no significant response to hormonal therapy. Lung biopsies are important for a diagnosis, but the size and location of the biopsy specimens greatly affect the diagnostic reliability. |
本系統中英文摘要資訊取自各篇刊載內容。