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相關文獻
- Concurrent Hepatic and Ruptured Renal Angiomyolipoma in Tuberous Sclerosis Complex
- 結節性硬化症
- 結節性硬化症(Tuberous Sclerosis)之臨床與病理學診斷
- The Management Strategy of Renal Angiomyolipoma
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- Treatment of the Facial Lesions in Tuberous Sclerosis--A Case Report
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- 顱內結節性硬化症之放射線徵象:強調電腦斷層造影之所見
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題 名 | Concurrent Hepatic and Ruptured Renal Angiomyolipoma in Tuberous Sclerosis Complex=結節性硬化症同時併發肝臟及破裂之腎臟血管肌脂肪瘤 |
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作 者 | 趙政漢; 林進耀; 陳肇長; 陳國書; | 書刊名 | 長庚醫學 |
卷 期 | 27:9 民93.09 |
頁 次 | 頁696-700 |
分類號 | 415.74 |
關鍵詞 | 結節性硬化症; 肝臟和腎臟血管肌脂肪瘤; Angiomyolipoma; Tuberous sclerosis; Liver; Kidney; |
語 文 | 英文(English) |
中文摘要 | 肝臟或腎臟的血管肌脂肪是結節性硬化症的臨床表現之一。然而,結節性硬化症同時發生肝臟及腎臟的血管肌脂肪瘤是一個罕見的疾病。越大的腎臟的血管肌脂肪瘤其破裂的機率也越大。在此我們報告同特發生肝臟及腎臟的血管肌脂肪瘤一個結節性硬化症病例,一開始是以腫瘤疲裂的急性腹痛為表現。腹部電腦斷層顯示肝臟與雙側腎臟有多發性的腫瘤。因為腫瘤破裂,右側腎臟被切除。同時肝臟切片初始診斷為壞死組織。然而使用HMB-45染色的免疫活性測定診斷為血管肌脂肪。我們所強調病理學家與臨床醫師應考慮到結節性硬化症可能併發肝臟或腎臟血管肌脂肪瘤。為了避兔不適當的診斷,建議使用HMB-45的免疫組織化學染色。 |
英文摘要 | Angiomyolipoma of the liver or kidney is one of the clinical manifestations of tuberous sclerosis complex. However, concurrence of angiomyolipoma in both liver and kidney associated with tuberous sclerosis complex is a rare entity. Renal angiomyolipomas with large aneurysms confer a higher probability of rupture as compared to small aneurysms. Herein, we document a case of tuberous sclerosis coexisting with hepatic and renal angiomyolipoma in a 37 year-old woman who presented with an acute abdomen due to ruptured tumbor. Computed tomography of the abdomen revealed multiple tumors over the bilateral kidneys and liver. A right nephrectomy was performed. During surgery, a live biopsy was performed which a preliminary diagnosis of necrosis was established. However, immunoreactivitystaining using monoclonal antibody HMB-45 (Human Melanoma, Black) led to the final diagnosis of angiomyolipoma. We emphasized that pathologists and clinicians should be aware that cases of tuberous sclerosis complex may be associated with renal and hepatic angiomyolipoma. To avoid an inappropriate diagnosis, before diagnosing liver necrosis, immunohistochemical staining for HMB-45 is recommended. |
本系統中英文摘要資訊取自各篇刊載內容。