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

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

Maciej Mach  1 , Karol Maciejewski  1 , Tomasz Ostrowski  1 , Rafał Maciąg  2 , Michał Sajdek  2 , Zbigniew Gałązka  1
Affiliations
Case Reports

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

Maciej Mach et al. Cureus. .

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.

Keywords: embolization; renal angiomyolipoma; renal intervention; renal tumor; tuberous sclerosis complex.

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Conflict of interest statement

Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. Multiple focal lesions in the right kidney (red arrow) and left kidney (yellow arrow)
Figure 2
Figure 2. A ruptured lesion in the right kidney (black arrow)
Figure 3
Figure 3. AML lesions in the right kidney during angiography (black arrows)
Figure 4
Figure 4. Right kidney nephrogram after embolization of three AML lesions and a visible lower pole AML lesion (black arrow)
AML: Angiomyolipoma
Figure 5
Figure 5. AML lesion in the left kidney during angiography (black arrow)
AML: Angiomyolipoma
Figure 6
Figure 6. Embolization of the AML lesion in the lower pole of the left kidney using Onyx-18
AML: Angiomyolipoma
Figure 7
Figure 7. Embolized lesion in the lower pole of the left kidney (black arrow)
Figure 8
Figure 8. Embolization of the AML lesion in the lower pole of the right kidney using Onyx-18
AML: Angiomyolipoma
Figure 9
Figure 9. Embolized lesion in the lower pole of the right kidney (black arrow)
See this image and copyright information in PMC

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