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

结节性硬化症患者双侧血管平滑肌脂肪瘤破裂的血管内治疗

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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.

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