Background: Giant angiomyolipoma is usually associated with genetic syndromes and complications (spontaneous rupture and bleeding, hematuria, hypertension) and mass-related symptoms (flank and abdominal pain).
Case Presentation: We present a case of a 20-year-old woman suffering from tuberous sclerosis who was referred to our hospital with a giant angiomyolipoma causing abdominal pain. A contrast-enhanced computed tomography showed a left angiomyolipoma, measuring 28 cm × 17 cm × 27 cm. After a multidisciplinary team discussion, the patient was submitted for a nephrectomy. Percutaneous temporary occlusion of the main renal artery was achieved through an endovascular balloon catheter. Through the balloon catheter guidewire, 2,500 IU of heparin was infused to reduce the risk of tumor vein thrombosis and venous embolism. This allowed a safe kidney manipulation through a left thoracoabdominal approach. The postoperative course was uneventful. Pathology showed a 40 cm × 30 cm × 9 cm and 10 kg AML. One year after surgery, the patient is on follow-up, and her estimated glomerular filtration is 120.5 ml/min/1.73 m.
Conclusion: The present case showed that the endovascular control of the main renal artery could be considered a useful approach to safely managing huge renal masses when renal hilar control is expected to be very difficult.
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http://dx.doi.org/10.3389/fsurg.2022.955932 | DOI Listing |
Diseases
December 2024
Department of Pediatrics, Dokkyo Medical University, Tochigi 321-0293, Japan.
Background: Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by mutations in the TSC1 and TSC2 genes, leading to the dysregulation of the mammalian target of rapamycin (mTOR) pathway. This dysregulation results in the development of benign tumors across multiple organ systems and poses significant neurodevelopmental challenges. The clinical manifestations of TSC vary widely and include subependymal giant cell astrocytomas (SEGAs), renal angiomyolipomas (AMLs), facial angiofibromas (FAs), and neuropsychiatric conditions such as autism spectrum disorder (ASD).
View Article and Find Full Text PDFFront Med (Lausanne)
December 2024
Department of Medicine, Service of Nephrology, Fribourg State Hospital, Fribourg, Switzerland.
Aim Of The Study: Tuberous sclerosis complex (TSC) is a genetic and multisystemic disorder that affects between 1/6'000 and 1/10'000 of newborns. Clinical criteria and/or genetic analysis establish the diagnosis. The mechanistic target of rapamycin (mTOR) inhibitors everolimus or sirolimus reduce the severity of several TSC-related clinical traits.
View Article and Find Full Text PDFUrol Case Rep
January 2025
Department of Histopathology, Mt Meru Regional Referral Hospital, P.O Box 3092, Arusha, Tanzania.
Unlabelled: Renal angiomyolipoma is an uncommon benign tumor that mostly affects adults and is highly associated with tuberous sclerosis. It's very uncommon for it to affect adolescents. In this case, a 17-year-old male with abdominal pain and distension was diagnosed with sporadic giant renal angiomyolipoma.
View Article and Find Full Text PDFTrop Doct
December 2024
Professor, Department of Pulmonary Medicine, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
Angiomyolipomas are the benign tumours of the kidney which occur either sporadically or in association with tuberous sclerosis complex (TSC). Symptoms typically develop with an increase in the size of the lesion. We present a clinical image of a patient with giant renal angiomyolipomas who had other clinical features of TSC (facial angiofibromas, periungual fibroma, subependymal nodules in the brain, and lung cysts).
View Article and Find Full Text PDFAsian J Surg
November 2024
Department of Urology, Kweichow Moutai Hospital, Renhuai, Guizhou, China. Electronic address:
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