结节性硬化症肾血管平滑肌脂肪瘤的治疗:一个病例系列。

IF 0.8 Q4 PERIPHERAL VASCULAR DISEASE
Ganesh G Gowda, Maureen Prativa Tigga, Rashmi Hosahalli Sreenath
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引用次数: 0

摘要

肾血管平滑肌脂肪瘤是由血管、脂肪组织和平滑肌组成的良性肿瘤,约占结节性硬化症患者的70%至80%。小于4厘米的血管平滑肌脂肪瘤通常无症状,而较大的血管平滑肌脂肪瘤可引起腰痛、贫血和血尿。与散发性血管平滑肌脂肪瘤相反,结节性硬化症相关的血管平滑肌脂肪瘤通常在年轻时出现,是多中心和大的,并且具有更高的危及生命的出血风险。治疗策略包括选择性肿瘤栓塞、严重病例的肾切除术和依维莫司等药物治疗。尽管结节性硬化症和肾血管平滑肌脂肪瘤之间存在相关性,并且文献中描述了它们的高并发症发生率,但临床实践中的疾病意识仍然很低。本病例系列描述了三例结节性硬化症相关肾血管平滑肌脂肪瘤患者选择性动脉栓塞治疗的管理和结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Management of Renal Angiomyolipomas in Tuberous Sclerosis: A Case Series.

Renal angiomyolipomas, benign tumors composed of blood vessels, adipose tissue, and smooth muscle, affect approximately 70% to 80% of patients with tuberous sclerosis. Angiomyolipomas smaller than 4 cm are usually asymptomatic, whereas larger ones can cause lumbar pain, anemia, and hematuria. Contrary to its sporadic counterparts, tuberous sclerosis-associated angiomyolipomas often present at a young age, are multicentric and large, and carry a higher risk of life-threatening hemorrhage. Therapeutic strategies include selective tumor embolization, nephrectomy for severe cases, and medical treatment such as everolimus. Despite a correlation between tuberous sclerosis and renal angiomyolipomas and their described high complication rates in literature, disease awareness in clinical practice remains low. This case series describes the management and outcomes of three patients with tuberous sclerosis-associated renal angiomyolipomas treated with selective arterial embolization.

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来源期刊
CiteScore
1.10
自引率
11.10%
发文量
29
审稿时长
17 weeks
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