Fat-poor renal angiomyolipoma in a patient with a end-stage kidney
Fat-poor renal angiomyolipoma in a patient with a end-stage kidney
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终末期肾脏患者的低脂肪肾血管平滑肌脂肪瘤
DOI：
10.1007/s13691-013-0114-x
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发表时间：
2014
期刊：
影响因子：
--
通讯作者：
Funyu T
中科院分区：
文献类型：
--
作者：
Murasawa H;Koie T;Hatakeyama S;Hashimoto Y;Yoneyama T;Saitoh H;Ohyama C;Funyu T
终末期肾脏血管平滑肌脂肪瘤是非常罕见的。血管平滑肌脂肪瘤与肾细胞癌的鉴别很重要，因为血管平滑肌脂肪瘤通常不需要手术切除。诊断为血管平滑肌脂肪瘤是通过在肾脏实质性肿块内显示大量脂肪。当血管平滑肌脂肪瘤含有大量的内部脂肪时，诊断并不困难。然而，如果血管平滑肌脂肪瘤含有少量脂肪，在CT扫描上检测可能具有挑战性。当怀疑肾脏肿块中有少量脂肪时，薄层CT平扫结合像素分析是确认脂肪存在的最敏感的检查。少数血管平滑肌脂肪瘤不含肉眼可见的脂肪，影像学上很难与肾脏肿瘤鉴别。一名患有慢性肾衰竭的52岁女性被收住到Oyokyo Kidney Research Institute广崎医院接受血液透析诱导。筛查计算机断层扫描显示左肾有一个增强型肿瘤，无脂肪衰减（大小3 × 2.5 cm）。基于这些发现，我们诊断病人为肾细胞癌，并施行根治性左肾切除术。然而，肿瘤的组织学检查显示存在血管瘤、肌瘤和脂肪瘤成分。此外，免疫组织学检查显示，肿瘤细胞的平滑肌肌动蛋白，结蛋白，和HMB-45强阳性。基于这些发现，我们诊断为终末期肾脏患者的脂肪贫乏型血管平滑肌脂肪瘤。诊断肾血管平滑肌脂肪瘤是具有挑战性的，当肿瘤含有少量的脂肪。术前仅根据横断面影像学检查可能难以区分脂肪贫乏型血管平滑肌脂肪瘤和肾细胞癌，尤其是在终末期肾脏患者中。
Angiomyolipoma in end-stage kidneys is very rare. The differentiation of an angiomyolipoma from a renal cell carcinoma is important because angiomyolipomas do not usually need to be surgically removed. The diagnosis of an angiomyolipoma is made by demonstrating bulky fat within a solid renal mass. When angiomyolipomas contain a large amount of internal fat, the diagnosis is not challenging. However, if the angiomyolipoma contains a small amount of fat, it can be challenging to detect on CT scanning. When a small amount of fat is suspected in a renal mass, an unenhanced CT examination with thin sections combined with a pixel analysis is the most sensitive test to confirm the presence of fat. A small number of angiomyolipomas do not contain macroscopic fat, and the imaging differentiation from a renal neoplasm is difficult. A 52-year-old woman with chronic renal failure was admitted to Oyokyo Kidney Research Institute Hirosaki Hospital for induction of hemodialysis. Screening computed tomography revealed an enhancing tumor without fat attenuation (size 3 × 2.5 cm) in the left kidney. Based on these findings, we diagnosed the patient with renal cell carcinoma and performed a radical left nephrectomy. However, histological examination of the tumor revealed the presence of angiomatous, myomatous, and lipomatous components. Furthermore, immunohistological examinations showed that the tumor cells were strongly positive for smooth muscle actin, desmin, and HMB-45. Based on these findings, we diagnosed fat-poor angiomyolipoma in a patient with end-stage kidneys. Diagnosing renal angiomyolipomas is challenging when the tumor contains a minimal amount of fat. It may be difficult to distinguish between fat-poor angiomyolipoma and renal cell carcinoma preoperatively based on cross-sectional imaging findings alone, especially in patients with end-stage kidneys.