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结节性硬化症中肾血管平滑肌脂肪瘤的管理:病例系列

Management of Renal Angiomyolipomas in Tuberous Sclerosis: A Case Series.

作者信息

Gowda Ganesh G, Tigga Maureen Prativa, Sreenath Rashmi Hosahalli

机构信息

Department of Vascular Surgery, JSS Academy of Higher Education and Research, Mysuru, India.

Department of Obstetrics and Gynecology, JSS Academy of Higher Education and Research, Mysuru, India.

出版信息

Vasc Specialist Int. 2024 Dec 16;40:40. doi: 10.5758/vsi.240039.

Abstract

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.

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70%至80%的结节性硬化症患者会受到影响。小于4厘米的血管平滑肌脂肪瘤通常无症状,而较大的肿瘤可引起腰痛、贫血和血尿。与散发性血管平滑肌脂肪瘤不同,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,多为多中心且体积较大,并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以及依维莫司等药物治疗。尽管结节性硬化症与肾血管平滑肌脂肪瘤之间存在关联,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认知度仍然较低。本病例系列描述了3例接受选择性动脉栓塞治疗的结节性硬化症相关肾血管平滑肌脂肪瘤患者的治疗及预后情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d19b/11646834/d677800af958/vsi-40-40-f1.jpg

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