Renal Angiomyolipoma
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2021 ◽  
Balint Botz

2021 ◽  
Vol 15 (1) ◽  
H. Bouaziz ◽  
M. Ghalleb ◽  
N. Tounsi ◽  
N. Riahi ◽  
H. Bouzaiene ◽  

Abstract Introduction Renal angiomyolipoma is considered a benign mesenchymal tumor composed of fat, smooth muscle, and blood vessels. It represents 1–3% of solid renal tumors. Despite this tumor’s benignity, it can be aggressive with a locoregional extension. Case report A 41-year-old north African caucasian woman consulted with chief complaints of right lower back pain with no hematuria and no urinal sign. Thoracic-abdominopelvic contrast-enhanced computed tomography showed a right inferior polar heterogeneous renal mass complicated with venous thrombus ascending to the right atrium level. The patient underwent radical nephrectomy under extracorporeal circulation and direct supervision of the fatty thrombus at the right atrium level. The postoperative period was uneventful. The final histologic examination was concordant with renal angiomyolipoma. Conclusion Renal angiomyolipoma is the most common benign kidney tumor. Despite its benignity, it can be associated with lethal complications such as hemorrhage, and it can also show signs of local extension mimicking malignant tumors. The cornerstone of the treatment remains surgery.

Sharifah NurDurrah Binti Syed Mudzhar ◽  
Mohd Yusran Othman

Tuberous Sclerosis Complex (TSC) is a rare neuro-cutaneous disorder that is associated with the development of benign hamartomas including renal angiomyolipoma (RAML). TSC associated RAML are usually asymptomatic, but it carries a life-threatening bleeding risk. We are sharing a case of a 5-year-old girl who was diagnosed to have TSC with associated subependymal giant cell astrocytoma, cardiac rhabdomyoma and autism. She presented with a history of worsening abdominal distension over 3 weeks duration and clinically noted to be pale with a ballotable left flank mass. Ultrasound and CT scan found to have multiple RAML in both kidneys with a huge mass on the left side. The mass represented a huge RAML (8cm) with aneurysmal formation with suspicion of intratumoral bleeding. The option of conservative management with mammalian target of rapamycin inhibitor followed with partial nephrectomy has been questioned with its life-threatening risk of bleeding and inability to do biopsy to rule out the possibility of renal cell carcinoma. Decision for nephrectomy was then made clearer following a MAG-3 scan which revealed only 11% differential function of the left kidney. She underwent a total left nephrectomy uneventfully and intraoperatively noted to have an enlarging lesion as compared to the previous imaging; 15cm in largest diameter. Histopathological finding was consistent with multifocal angiomyolipoma with intratumoral haematoma. Decision for nephrectomy in TSC-associated RAML need to be justified carefully in view of its risk of losing the contralateral kidney following the disease progression which may end up with life-long renal replacement therapy.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S26

2021 ◽  
Vol 10 (18) ◽  
pp. 4062
François-Victor Prigent ◽  
Kévin Guillen ◽  
Pierre-Olivier Comby ◽  
Julie Pellegrinelli ◽  
Nicolas Falvo ◽  

Selective arterial embolization (SAE) for renal angiomyolipoma (rAML) is effective to treat or prevent bleeding. We report our experience using a cyanoacrylate–Lipiodol mixture. We performed a single-center retrospective review of all rAMLs embolized with cyanoacrylate glue between July 2014 and June 2020. Demographics, tuberous sclerosis complex (TSC) status, clinical presentation, angiography features, and follow-up data were recorded. Pre- and post-procedure rAML sizes and volumes were estimated from computed tomography (CT) or magnetic resonance imaging (MRI) studies. Kidney function was assessed before and after the procedure. We identified 24 patients (22 females and 2 males, mean age 51 years) treated for 27 AMLs, either prophylactically (n = 20) or as an emergency (n = 4). Technical success was achieved for 25/27 AMLs; two patients, each with a single AML, required nephrectomy and repeated embolization, respectively. Major complications occurred in three patients and minor complications such as postembolization syndrome in 15 patients. AML volume reduction after embolization was 55.1% after a mean follow-up of 15 months (range, 1–72 months). Factors associated with greater volume reduction were a smaller percentage of fat (p = 0.001), larger initial rAML volume (p = 0.014), and longer follow-up (p = 0.0001). The mean creatinine level did not change after SAE. Embolization of rAMLs with a mixture of cyanoacrylate and Lipiodol is feasible, safe, and effective in significantly decreasing tumor volume.

2021 ◽  
Vol 8 ◽  
Maofeng Gong ◽  
Zhengli Liu ◽  
Haobo Su ◽  
Boxiang Zhao ◽  
Jie Kong ◽  

Purpose: Wunderlich syndrome (WS) with hypovolemic shock secondary to ruptured renal angiomyolipoma (rAML) represents an urgent condition. Hence, we reported our experience with transcatheter arterial embolization (TAE) using different embolic materials under this condition.Methods: This retrospective study consisted of 22 patients. Embolic materials including particles, microcoils, and liquid embolic agents were selectively used based on the decisions of interventional radiologists. Technical success was defined as the complete occlusion of bleeding vessels on the final renal angiogram. Clinical success was defined as the absence of re-hemorrhage needed for repeat endovascular or surgery treatment after TAE.Results: The articulated materials were used when WS presented without aneurysms; a combination of particulate materials and microcoils or Glubran 2 alone were used for WS with aneurysms. The technical success based on 24 episodes of TAEs in 22 patients was 100% (24 of 24). Repeat TAE was achieved in two patients with hemorrhages re-occurring two days after the initial embolization with microcoils alone. The clinical success was 90.9% (20 of 22). No nontarget embolization or embolization-related complications occurred during the TAE procedure. Of the patients, 27.3% (6 of 22) experienced minor complications of post-embolization syndrome (PES). During a median follow-up time of 34 months, no recurrent hemorrhage that required repeat endovascular or surgical treatment for hemostasis occurred.Conclusion: Urgent TAE with the selective use of different embolic materials is an effective alternative to control WS with hypovolemic shock secondary to ruptured rAML. The condition of presenting with or without aneurysms may determine the embolic materials employed.

2021 ◽  
Vol Volume 13 ◽  
pp. 707-716
Jarmila Kruseová ◽  
Barbora Gottfriedová ◽  
Andrea Zichová ◽  
Karel Švojgr ◽  
Petr Hošek ◽  

Urology ◽  
2021 ◽  
Sidney I. Roberts ◽  
Sanam Ladi-Seyedian ◽  
Siamak Daneshmand

2021 ◽  
Vol 14 (8) ◽  
pp. e243380
Ramon Jr Bagaporo Larrazabal ◽  
Harold Henrison Chang Chiu ◽  
Dennis L Sacdalan

A 28-year-old woman came for non-traumatic right flank pain with hypotension and right flank mass. She had multiple hyperpigmented skin papules located on the centre area of her face, and angiomas on her toes. She was anaemic and had a blood transfusion on top of aggressive fluid resuscitation. Abdominal CT showed bilaterally enlarged kidneys and fluid collection in the right perirenal space (haemorrhage). The consideration was an angiomyolipoma in spontaneous perinephric haemorrhage. We considered tuberous sclerosis complex (TSC) and did genetic testing. Results revealed mutations in the TSC2 gene, consistent with the diagnosis of TSC. No immediate surgical plans were considered at that time. She opted to be discharged against medical advice and was scheduled for a close outpatient follow-up. The patient followed up after 2 weeks, already on sirolimus 2 mg once daily. She reported improved overall well-being and a decrease in the flank mass size.

Niharika Neela ◽  
Hula Taha ◽  
Jaya Kala

2021 ◽  
Vol 15 (1) ◽  
Yang Zhao ◽  
Hao Guo ◽  
Wenda Wang ◽  
Guoyang Zheng ◽  
Zhan Wang ◽  

Abstract Objective Tuberous sclerosis complex (TSC) is a rare autosomal dominant disease characterized by lesions throughout the body. Our previous study showed the abnormal up-regulation of miRNAs plays an important part in the pathogenesis of TSC-related renal angiomyolipoma (TSC-RAML). circRNAs were known as important regulators of miRNA, but little is known about the circRNAs in TSC-RAMLs. Methods Microarray chips and RNA sequencing were used to identify the circRNAs and mRNAs that were differently expressed between the TSC-RAML and normal kidney tissue. A competitive endogenous RNA (ceRNA) regulatory network was constructed to reveal the regulation of miRNAs and mRNAs by the circRNAs. The biological functions of circRNA and mRNA were analyzed by pathway analysis. Microenvironmental cell types were estimated with the MCP-counter package. Results We identified 491 differentially expressed circRNAs (DECs) and 212 differentially expressed genes (DEGs), and 6 DECs were further confirmed by q-PCR. A ceRNA regulatory network which included 6 DECs, 5 miRNAs, and 63 mRNAs was established. Lipid biosynthetic process was significantly up-regulated in TSC-RAML, and the humoral immune response and the leukocyte chemotaxis pathway were found to be down-regulated. Fibroblasts are enriched in TSC-RAML, and the up-regulation of circRNA_000799 and circRNA_025332 may be significantly correlated to the infiltration of the fibroblasts. Conclusion circRNAs may regulate the lipid metabolism of TSC-RAML by regulation of the miRNAs. Fibroblasts are enriched in TSC-RAMLs, and the population of fibroblast may be related to the alteration of circRNAs of TSC-RAML. Lipid metabolism in fibroblasts is a potential treatment target for TSC-RAML.

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