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題 名 | 淋巴血管平滑肌瘤臨床表現腹腔腫塊合併乳糜腹水--病例報告及文獻回顧=Lymphangioleiomyomatosis Clinical Presented with Abdominal Tumor and Chylous Ascites--A Case Report and Literatures Review |
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作 者 | 蔡偉宏; 蕭富源; 吳佩儒; 何上芸; 蔡政宏; 陳正雄; | 書刊名 | 中華民國重症醫學雜誌 |
卷 期 | 12:4 2011.12[民100.12] |
頁 次 | 頁205-211 |
分類號 | 416.264 |
關鍵詞 | 淋巴血管平滑肌瘤; 乳糜腹水; Lymphagiomyomatosis; Chylous ascites; HMB-45; |
語 文 | 中文(Chinese) |
中文摘要 | 淋巴血管平滑肌瘤( LAM)是一種罕見的疾病,病因不明,患者幾乎完全於生育年齡婦女,偶爾在停經後婦女。淋巴管平滑肌瘤起源於瀰漫性增生的平滑肌細胞。患者常呈現進行性呼吸困難或復發性氣胸,乳糜胸腹腔腫瘤或出血。淋巴管阻塞可導致後腹腔囊性腫塊或乳糜胸。我們報告一例案例,一位女性病患因下腹部脹痛就醫,身體檢查發現下腹部腫瘤,腹部電腦斷層掃描顯示後腹腔腫塊同時合併腹水,腹水抽吸資料顯示乳糜腹水,腹水顯示三酸甘油酯 (TG)高達 1848毫克 /百毫升。腹腔鏡腫塊切除手術,病理免疫組織化學研究發現平滑肌束,人類黑色素瘤單株抗體 HMB-45 (+),雌激素受體 estrogen receptor (-),細胞角蛋白 cytokeratin (-),CD-34 (-)。胸部電腦斷層掃描顯示整個肺部有許多薄壁的囊腫,因此診斷肺部併肺外的淋巴管平滑肌瘤。 對於生育年齡婦女,發現腹腔腫瘤合併乳糜腹水必須考慮淋巴管平滑肌瘤。電腦斷層掃描、病理組織切片、免疫組織化學染色特定的平滑肌成分;平滑肌肌動蛋白 (smooth muscle actin),結蛋白 (Desmin),或 HMB-45可以協助診斷。 |
英文摘要 | A 43-year-old Taiwanese female patients with abdominal distension and lower abdominal lump for 4-5 months, because abdominal pain for few days so visited clinic for medical survey, physical examination found that the lower abdomen groin lump, slightly lower abdominal tenderness, no rebound pain, no weight loss, no dyspnea or chest pain. Normal chest X-ray, WBC: 6500, Hb: 10.5 Platelet: 350000 / cmm. Abdominal CT scan revealed a retroperitoneal mass (6.2X2.8X4 cm) concomitant lymph nodes and ascites. Abdominal aspiration showed that mild blood contain milky like chylous ascites. Ascites analysis showed that red blood cells: 203,000 / cmm, white blood cells: 1350/cmm, lymphocyte ratio: 98%, ascites triglyceride level up to 1848mg/dl, peripheral blood triglyceride level of 149mg/dl. Another test CEA: 0.8μg/ml (normal <5), CA-125: 607μg/ml (normal <35), CA 19-9: 3.52μg/ml (normal <37). Ascites cytology showed not malignant cells, acid fast stain and TB culture of ascites all negative. Then patient arranged abdominal tumor surgery, pathology found spindle smooth muscle bundles, special staining immunohistochemical study HMB-45 (+). Arrange chest CT scan showed the lung has many thin-walled cyst, so diagnosis of pulmonary and extra-pulmonary lymphagiomyomatosis . |
本系統中英文摘要資訊取自各篇刊載內容。