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多发性肾血管平滑肌脂肪瘤。病例报告。

Multiple renal angiomyolipomata. A case report.

作者信息

Kołacz Jacek, Irzyk Małgorzata, Urbańczyk-Zawadzka Małgorzata

机构信息

Interdyscyplinarny Zespół Pracowni Diagnostyki Obrazowej, Ośrodek Diagnostyki, Prewencji i Telemedycyny, Krakowski Szpital Specjalistyczny im. Jana Pawła II, Kraków, Polska.

出版信息

J Ultrason. 2012 Sep;12(50):349-53. doi: 10.15557/JoU.2012.0020. Epub 2012 Sep 30.

Abstract

We present a 76-year-old patient with multiple renal angiomylipoma in one kidney. The patient had not been having any urinary tract symptoms, the lesions in kidney were visualized in US examination performed because of cholelithiasis suspicion. Angiomyolipoma is included to the benign tumors of hamartoma group and its growth is related to the hormonal activity of the organism. Multiple renal angiomyolipomata, occurring very rarely, are usually related to the tuberous sclerosis syndrome (Bourneville-Pringle disease). The nature of this disease is the occurrence of multiple hamartoma type tumors in the skin, brain, kidneys, heart, bones, lungs and eyes. Small renal angiomyolipomata are asymptomatic and are usually accidentally diagnosed during imaging examinations, big ones may be the reason of significant ailments. Among severe, life threatening complications of renal angiomyolipoma one can number bleeding from the tumor. Bleeding risk depends on the tumor diameter and significantly increases in tumors of the diameter above 4 cm. Computed tomography is an imaging method recommended for the assessment of hemorrhagic complications in angiomyolipoma. The check-up frequency depends on the tumor diameter - in tumors smaller than 4 cm the examination is performed once yearly, in tumors greater than 4 cm - every 6 months. In the treatment of hemorrhagic complications of angiomyolipoma, a surgical treatment (partial or radical nephrectomy) or renal vessel embolization is used. Renal arteriography with embolization is an important therapeutic method to control the bleeding and to avoid surgery.

摘要

我们报告一例76岁患者,其一侧肾脏存在多发肾血管平滑肌脂肪瘤。该患者此前无任何尿路症状,因怀疑患有胆结石而进行的超声检查发现了肾脏中的病变。血管平滑肌脂肪瘤属于错构瘤组的良性肿瘤,其生长与机体的激素活性有关。多发肾血管平滑肌脂肪瘤极为罕见,通常与结节性硬化综合征(布尔内维尔-普林格尔病)有关。这种疾病的本质是在皮肤、大脑、肾脏、心脏、骨骼、肺和眼睛中出现多发性错构瘤样肿瘤。较小的肾血管平滑肌脂肪瘤无症状,通常在影像学检查时偶然被诊断出来,较大的肿瘤可能是严重疾病的原因。肾血管平滑肌脂肪瘤严重的、危及生命的并发症之一是肿瘤出血。出血风险取决于肿瘤直径,直径超过4厘米的肿瘤出血风险显著增加。计算机断层扫描是评估血管平滑肌脂肪瘤出血并发症的推荐成像方法。检查频率取决于肿瘤直径——直径小于4厘米的肿瘤每年检查一次,直径大于4厘米的肿瘤每6个月检查一次。在治疗血管平滑肌脂肪瘤的出血并发症时,采用手术治疗(部分或根治性肾切除术)或肾血管栓塞术。肾动脉造影栓塞术是控制出血和避免手术的重要治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a41f/4582528/5c67daf4d2b4/JoU-2012-0020-g001.jpg

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