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结节性硬化症患者双侧破裂血管平滑肌脂肪瘤的血管内治疗

Endovascular Treatment of a Bilateral, Ruptured Angiomyolipoma in a Patient With Tuberous Sclerosis Complex.

作者信息

Mach Maciej, Maciejewski Karol, Ostrowski Tomasz, Maciąg Rafał, Sajdek Michał, Gałązka Zbigniew

机构信息

Department of General, Vascular, Endocrine and Transplant Surgery, Medical University of Warsaw, Warsaw, POL.

2nd Department of Clinical Radiology, Medical University of Warsaw, Warsaw, POL.

出版信息

Cureus. 2024 Aug 5;16(8):e66200. doi: 10.7759/cureus.66200. eCollection 2024 Aug.

DOI:10.7759/cureus.66200
PMID:39105203
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11299878/
Abstract

A renal angiomyolipoma (AML) is a rare, usually benign tumor consisting of smooth muscle cells, abnormal blood vessels, and fat tissue. Although AMLs are often asymptomatic, they can present with flank pain, hematuria, and a palpable mass in the abdomen. A significant complication involves rupture and hemorrhage into the retroperitoneal cavity, which can be life-threatening. The treatment approach has evolved from surgical removal to more conservative management, such as nephron-sparing embolization and mammalian target of rapamycin (mTOR) inhibitors for tuberous sclerosis complex (TSC)-associated AML. In March 2024, a 36-year-old female patient diagnosed with TSC was admitted to our department and underwent several endovascular embolizations after a life-threatening hemorrhage from a ruptured multilocular AML. The treatment was successful, with complete exclusion of the AMLs from circulation and without any complications during the postoperative period. This case emphasizes the effectiveness of selective arterial embolization using the Onyx liquid embolic system in managing AMLs and highlights the importance of preserving renal function. Methods used in AML diagnosis include ultrasound and computed tomography scans, with magnetic resonance imaging and biopsy recommended in difficult cases. Treatment depends on aspects such as tumor size, symptoms, and patient's general condition, with options ranging from active surveillance for small, asymptomatic AMLs to more invasive procedures for larger, symptomatic tumors. The main goal is to minimize symptoms and preserve renal function.

摘要

肾血管平滑肌脂肪瘤(AML)是一种罕见的、通常为良性的肿瘤,由平滑肌细胞、异常血管和脂肪组织组成。尽管AML通常无症状,但可表现为胁腹痛、血尿和腹部可触及肿块。一个重要的并发症是肿瘤破裂并出血进入腹膜后腔,这可能危及生命。治疗方法已从手术切除演变为更保守的治疗,如保留肾单位的栓塞术以及用于治疗结节性硬化症(TSC)相关AML的哺乳动物雷帕霉素靶蛋白(mTOR)抑制剂。2024年3月,一名诊断为TSC的36岁女性患者因多房性AML破裂导致危及生命的出血而入住我科,并接受了多次血管内栓塞治疗。治疗成功,AML完全从循环中排除,术后无任何并发症。该病例强调了使用Onyx液体栓塞系统进行选择性动脉栓塞治疗AML的有效性,并突出了保留肾功能的重要性。AML诊断中使用的方法包括超声和计算机断层扫描,在疑难病例中推荐使用磁共振成像和活检。治疗取决于肿瘤大小、症状和患者的一般状况等因素,选择范围从小的无症状AML的主动监测到较大的有症状肿瘤的更具侵入性的手术。主要目标是尽量减轻症状并保留肾功能。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/aa8780656846/cureus-0016-00000066200-i09.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/034e217b7125/cureus-0016-00000066200-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/85c323b0e5fb/cureus-0016-00000066200-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/fc64fc52980f/cureus-0016-00000066200-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/a057057c4207/cureus-0016-00000066200-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/91faf72f29f7/cureus-0016-00000066200-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/b3234dc07a99/cureus-0016-00000066200-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/3edc458f69c7/cureus-0016-00000066200-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/8c5ec5f1f105/cureus-0016-00000066200-i08.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/aa8780656846/cureus-0016-00000066200-i09.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/034e217b7125/cureus-0016-00000066200-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/85c323b0e5fb/cureus-0016-00000066200-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/fc64fc52980f/cureus-0016-00000066200-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/a057057c4207/cureus-0016-00000066200-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/91faf72f29f7/cureus-0016-00000066200-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/b3234dc07a99/cureus-0016-00000066200-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/3edc458f69c7/cureus-0016-00000066200-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/8c5ec5f1f105/cureus-0016-00000066200-i08.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccd4/11299878/aa8780656846/cureus-0016-00000066200-i09.jpg

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