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对4个月内快速生长且正在出血的小肾血管平滑肌脂肪瘤进行急诊栓塞治疗。

Emergency embolization of actively bleeding small renal angiomyolipoma with a fast growth in four months.

作者信息

Chiancone Francesco, Mirone Vincenzo, Imbimbo Ciro, Pucci Luigi, Meccariello Clemente, Fedelini Maurizio, Fedelini Paolo

机构信息

1 Urologic Clinic, University Federico II of Naples, Naples - Italy.

出版信息

Urologia. 2015 Apr-Jun;82(2):106-8. doi: 10.5301/uro.5000122. Epub 2015 Apr 21.

Abstract

Angiomyolipoma (AML) is the most common benign tumour of the kidney and is composed of smooth muscle cells, blood vessels and fat elements. AMLs are usually casually discovered when the kidneys are imaged for other reasons. Their first presentation is often a spontaneous retroperitoneal haemorrhage or bleeding into the urinary collection system, which can be life-threatening. The bleeding tendency is related to the irregular, aneurysmal, tortuous blood vessels that compose the tumor. The greatest risks for bleeding are tumour size and grade of the vascular component of the tumour. Moreover, the risk of bleeding is proportional to the size of the lesion (it is higher in AMLs with a diameter more than 4 cm). Shock due to a grave haemorrhage from AML rupture is called Wunderlich syndrome that is clinically characterized by the Lenk's triad: acute flank pain, a flank mass and hypovolemic shock.Other symptoms and signs may be present: haematuria, palpable mass, flank pain, urinary tract infections, renal failure or hypertension. Small AMLs usually require no therapy, although follow-up is recommended in order to follow its growth. Larger or symptomatic lesions can be electively embolized and/or resected with a partial nephrectomy. AMLs that present with retroperitoneal haemorrhage frequently require emergency embolization. We describe a case of an actively bleeding small renal AML, with a rapid growth, without any evidence for big aneurysmal vessels, who underwent an emergency embolization at our hospital.

摘要

血管平滑肌脂肪瘤(AML)是最常见的肾脏良性肿瘤,由平滑肌细胞、血管和脂肪成分组成。AML通常在因其他原因对肾脏进行成像检查时偶然被发现。其首次表现往往是自发性腹膜后出血或向泌尿系统内出血,这可能危及生命。出血倾向与构成肿瘤的不规则、动脉瘤样、迂曲的血管有关。出血的最大风险因素是肿瘤大小和肿瘤血管成分的分级。此外,出血风险与病变大小成正比(直径超过4cm的AML出血风险更高)。AML破裂导致严重出血引起的休克称为温德利希综合征,其临床特征为伦克三联征:急性胁腹痛、胁腹肿块和低血容量性休克。可能还会出现其他症状和体征:血尿、可触及的肿块、胁腹痛、尿路感染、肾衰竭或高血压。小的AML通常无需治疗,不过建议进行随访以观察其生长情况。较大的或有症状的病变可选择进行栓塞和/或通过部分肾切除术切除。出现腹膜后出血的AML通常需要紧急栓塞治疗。我们描述了一例在我院接受紧急栓塞治疗的活动性出血的小肾AML病例,该肿瘤生长迅速,未发现有大的动脉瘤样血管。

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