|Year : 2020 | Volume
| Issue : 2 | Page : 126-129
Giant renal angiomyolipoma managed by selective renal angioembolization: A unique case report
G Ajay Kumar1, P V Siva Prasad2, A Ravi Kumar2, A Sindhuri3, B S R Prasad Babu4
1 Department of Urology, Sentini Hospitals, Vijayawada, Andhra Pradesh, India
2 Cardiology, Sentini Hospitals, Vijayawada, Andhra Pradesh, India
3 Pulmonology, Sentini Hospitals, Vijayawada, Andhra Pradesh, India
4 Radiology, Sentini Hospitals, Vijayawada, Andhra Pradesh, India
|Date of Submission||19-Apr-2020|
|Date of Acceptance||19-Jun-2020|
|Date of Web Publication||11-Sep-2020|
Dr. G Ajay Kumar
Department of Urology, Sentini Hospitals, Vijayawada, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Renal angiomyolipomas (RMLs) can occur sporadically as solitary tumors or can be associated with tuberous sclerosis and present as bilateral multifocal disease. Modern radiographic imaging with ultrasonography (USG) or computed tomography (CT) scan can often diagnose this lesion based on its characteristic fat content. Even though it is a benign lesion, angiomyolipomas (AMLs) can be associated with symptoms, abdominal lump, and in some cases hemorrhage. Most can be managed with nephron-sparing approaches that include observation, angiographic embolization, or partial nephrectomy. The uniqueness of our article is that we successfully treated giant RML by angiography with selective renal angioembolization (RAE).
Keywords: Angiomyolipoma, embolization, kidney
|How to cite this article:|
Kumar G A, Prasad P V, Kumar A R, Sindhuri A, Babu B S. Giant renal angiomyolipoma managed by selective renal angioembolization: A unique case report. J Sci Soc 2020;47:126-9
|How to cite this URL:|
Kumar G A, Prasad P V, Kumar A R, Sindhuri A, Babu B S. Giant renal angiomyolipoma managed by selective renal angioembolization: A unique case report. J Sci Soc [serial online] 2020 [cited 2020 Sep 19];47:126-9. Available from: http://www.jscisociety.com/text.asp?2020/47/2/126/294792
| Introduction|| |
Angiomyolipoma (AML) is a benign tumor composed of mature or immature fat, thick-walled blood vessels and smooth muscle elements in varying proportions. There are two varieties of renal angiomyolipoma (RML), one associated with tuberous sclerosis (TS or Bourneville disease) and the other that appears as an isolated lesion. In the first case, RMLs are most often multiple, bilateral, symptomatic, and without female or male predominance. In the second case, RMLs are single, often asymptomatic, have a female predominance, and are typical of the fifth and sixth decades of life. The most common presentation of AML is an incidental finding, however, once a size of 4 cm is reached, symptoms may develop in up to 68%–80% of patients such as loin pain, hemorrhage, which may be life-threatening, or as a palpable mass.
Superselective arterial embolization (SAE) is a valuable treatment of RML. It is a minimally invasive intervention that can control bleeding in the majority of patients. Moreover, successful superselective embolization of the bleeding vessel preserves the function of the rest of the kidney parenchyma. Therefore, it was considered the management of choice of symptomatic AMLs in several studies ,,, The management of giant AMLs is a urological challenge. Different approaches were used for management of such lesions including conservative, nephrectomy,,,, and nephron-sparing surgery (NSS)., Reports of angioembolization in the management of giant AML are lacking. Few reports of embolotherapy included some patients with giant AMLs,,,, and only one study was reported including only cases of giant AMLs.
The risk of bleeding and surrounding tissue damage is proportional to the size of the lesion (diameter >4 cm). AMLs may also be associated with palpable mass, flank pain, urinary tract infections, hematuria, renal failure, hypertension, and, rarely, renal vein and/or inferior vena cava thrombosis. AMLs found incidentally are usually small and so require no therapy. Lesions that present with retroperitoneal hemorrhage often require emergency transarterial embolization as a lifesaving measure. Although embolization is effective for this purpose, some authors report a significant percentage of recurrent hemorrhage, recurrent symptoms, or inadequate tumor shrinkage after embolization.
| Case Report|| |
A 36-year-old female presented to our outpatient department services with a huge lump in the right side of the abdomen with decreased appetite. There was no prior relevant surgical history. She completed her family, with two children. Routine blood tests and complete urine examination were within normal limits. Ultrasonograghy (USG) abdomen and computed tomography (CT) were done, showing a giant right RML measuring 20 cm × 18 cm × 15 cm occupying the entire right side of the abdomen [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d. Because the patient is stable, options of its management were explained to the patient and their relatives.
|Figure 1: (a) USG abdomen: giant right renal angiomyolipoma. (b-d) Computed tomography urogram (sagittal, coronal, and axial views) showing giant right angiomyolipoma measuring 20 cm × 18 cm × 15 cm, displacing the kidney inferiorly. (e-h) Renal angiogram showing the accessory branch originating from the main right renal artery supplying the giant angiomyolipoma, embolization was performed with tightly calibrated, hydrogel microspheres. (i) Postarterial embolization, angiogram showing no blood flow into the accessory branch supplying the giant renal angiomyolipoma. (j) Postoperative USG abdomen showing decreased sine of the right renal angiomyolipoma to 6 cm × 5 cm × 4 cm|
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The patient was evaluated by a multidisciplinary team consisting of a urologist, a cardiologist, and a vascular surgeon. It was decided to utilize a combined interventional treatment, in order to reduce the risk of future hemorrhage, achieve a degree of tumor shrinkage, and minimize the adverse effects on the integrity and function of the solitary right kidney. SAE was performed first. Vascular access was gained through the right common femoral artery, with Seldinger technique, and after a flush aortogram, the right renal artery was selectively catheterized with 7 Fr renal double curve catheter. Check angiography showed the ectopic right kidney located in the midline with the hilum facing posterior superiorly. Large, heterogeneously dense, predominantly fat-attenuating lesion noted arising from the upper pole of the right kidney. The lesion is supplied by the right upper lobar branch of the main renal artery. The right kidney's upper pole branch was crossed with 0.014 cm × 190 cm BHW™ guide wire and exchanged with 4.0 Fr Slip-Cath Beacon Tip catheter. The upper pole branch embolization was done with 2-mm hilal embolization microcoils (02), 3-mm micronester embolization coil (01), and polyvinyl alcohol (500) particles with tightly calibrated, hydrogel microspheres [Figure 1]e, [Figure 1]f, [Figure 1]g, [Figure 1]h.
Postembolization renal angiography showed no blood flow into the branch supplying the renal AML [Figure 1]i. Post embolization, the patient was stable with no signs of renal infarction. With the exception of right flank pain, the procedure was well tolerated and the patient was discharged after 2 days.
The patient was on close follow-up for 3 years and on postoperative imaging USG, the size of AML has decreased in size to around 6 cm × 5 cm × 4 cm [Figure 1]j. No renal AML complaints from the patient were noted in the follow-up period.
| Discussion|| |
Renal AML is considered a benign kidney tumor with hamartomatous features. AML is composed of heterogeneous tissues, including blood vessels, adipose tissue, and smooth muscle, and may present as sporadic cases or in association with TS complex (TSC).
Renal AMLs are benign neoplasms composed of thick-walled blood vessels, smooth muscle, and adipose tissue in varying proportions. These lesions have been isolated in extrarenal locations, including the liver, lymph nodes, inferior vena cava, bladder, and spleen. Two phenotypes predominate. The overall female/male predominance is approximately 4:1. In the first phenotype, tumors present sporadically as a single unilateral renal lesion; the mean age of onset is in the fifth or sixth decades of life. The second phenotype, associated with TS, usually presents in the third decade of life as multifocal bilateral renal masses. The strong female prevalence suggests a hormonal component to tumor growth., This hypothesis is supported by documented cases of rapid AML growth during pregnancy and the identification of progesterone and estrogen receptors on smooth muscle nuclei of some AMLs using immunohistochemical staining.,
In the majority of cases, classic AML is easily diagnosed by recognition of fat tissue within the lesion, which appears hyperechoic on ultrasound, as an area of negative attenuation value on CT, and as an area of high signal intensity on T1-weighted images with signal loss on magnetic resonance imaging., Recognizing the fat component is, therefore, essential to rule out a diagnosis of malignant renal tumors such as renal cell carcinoma (RCC), as well as of lipomas, liposarcomas, and fat-containing RCCs. A percutaneous renal biopsy can be helpful in dubious cases.
Indications for the treatment of AML include intractable pain, hematuria, suspicion of malignancy, large-sized tumors, spontaneous ruptures, and radiographic imaging, suggestive of malignant lesions.
NSS has been used in the management of AML. Boorjian et al. reviewed 58 patients undergoing partial nephrectomy for renal AML and found that renal function was preserved and no patient required retreatment during a median follow-up of 8 years. Furthermore, NSS has been reported in the management of giant AML., Although a clear advantage of a surgical procedure such as partial nephrectomy is the elimination of the need for recurrent angiography and SAE, partial nephrectomy in such huge masses may be very difficult and any surgical intervention is likely to greatly reduce renal function. Furthermore, the characteristics of TSC-associated AML such as large size and multiplicity have limited the role of NSS.
Ramon et al. described his experience of treating by SAE containing AML in 41 patients with 48 kidneys with an average follow-up period of 4.8 years. Successful SAE was achieved in forty patients (91%), and avoidance of surgery was achieved in 96% of the kidneys. During follow-up, 98% of kidneys were preserved.
| Conclusion|| |
The present case was successfully managed by renal SAE for giant renal AML, without any major comorbid complications. A good expertise as a combined teamwork is needed in managing such cases. Patients with giant renal AML treated by SAE must be aware of the need for continued close disease surveillance. Long-term follow-up is needed in such cases.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Steiner MS, Goldman SM, Fishman EK, Marshall FF. The natural history of renal angiomyolipoma. J Urol 1993;150:1782-6.
Halpenny D, Snow A, McNeill G, Torreggiani WC. The radiological diagnosis and treatment of renal angiomyolipoma-current status. Clin Radiol 2010;65:99-108.
Lenton J, Kessel D, Watkinson AF. Embolization of renal angiomyolipoma: Immediate complications and long-term outcomes. Clin Radiol 2008;63:864-70.
Lee SY, Hsu HH, Chen YC, Huang CC, Wong YC, Wang LJ, et al
. Embolization of renal angiomyolipomas: Short-term and long-term outcomes, complications, and tumor shrinkage. Cardiovasc Intervent Radiol 2009;32:1171-8.
Ramon J, Rimon U, Garniek A, Golan G, Bensaid P, Kitrey ND, et al
. Renal angiomyolipoma: Long-term results following selective arterial embolization. Eur Urol 2009;55:1155-61.
Chick CM, Tan BS, Cheng C, Taneja M, Lo R, Tan YH, et al
. Long-term follow-up of the treatment of renal angiomyolipomas after selective arterial embolization with alcohol. BJU Int 2010;105:390-4.
Danforth TL, Lane BR, Novick AC. Conservative management of giant symptomatic angiomyolipomas in patients with the tuberous sclerosis complex. BJU Int 2007;100:794-7.
Nepple KG, Bockholt NA, Dahmoush L, Williams RD. Giant renal angiomyolipoma without fat density on CT scan: Case report and review of the literature. ScientificWorldJournal 2010;10:1334-8.
Nasir K, Ahmad A. Giant renal angiomyolipomas and pulmonary lymphangiomyomatosis. Saudi J Kidney Dis Transpl 2010;21:314-9.
] [Full text]
Panizzo V, Goi G, Moroni E, Micheletto G, Bruni B, Danelli P. Giant angiomyolipoma of the kidney not associated with tuberous sclerosis: A case report and review of the literature. Chir Ital 2009;61:507-513.
Katayama K, Hayashi T, Tsutahara K, Matsuoka A, Fujimoto N, Koide T. Bilateral giant renal angiomyolipoma following hemodialysis in a patient with tuberous sclerosis. Hinyokika Kiyo 2009;55:335-8.
Wahab S, Ahmad Khan R, Thapa M, Wahab A, Ahmad I. Giant angiomyolipoma associated with a dilated vessel prone to hemorrhage. Iran J Kidney Dis 2009;3:168-71.
Singla A, Chaitanya Arudra SK, Bharti N. Giant sporadic renal angiomyolipoma treated with nephron-sparing surgery. Urology 2009;74:294-5.
Lee W, Kim TS, Chung JW, Han JK, Kim SH, Park JH. Renal angiomyolipoma: Embolotherapy with a mixture of alcohol and iodized oil. J Vasc Interv Radiol 1998;9:255-61.
Ewalt DH, Diamond N, Rees C, Sparagana SP, Delgado M, Batchelor L, et al
. Long-term outcome of transcatheter embolization of renal angiomyolipomas due to tuberous sclerosis complex. J Urol 2005;174:1764-6.
Bishay VL, Crino PB, Wein AJ, Malkowicz SB, Trerotola SO, Soulen MC, et al
. Embolization of giant renal angiomyolipomas: Technique and results. J Vasc Interv Radiol 2010;21:67-72.
Li D, Pua BB, Madoff DC. Role of embolization in the treatment of renal masses. Semin Intervent Radiol 2014;31:70-81.
Murray TE, Doyle F, Lee M. Transarterial embolization of angiomyolipoma: A systematic review. J Urol 2015;194:635-9.
de Luca S, Terrone C, Rossetti SR. Management of renal angiomyolipoma: A report of 53 cases. BJU Int 1999;83:215-8.
Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyolipoma. J Urol 2002;168:1315-25.
Stillwell TJ, Gomez MR, Kelalis PP. Renal lesions in tuberous sclerosis. J Urol 1987;138:477-81.
Oesterling JE, Fishman EK, Goldman SM, Marshall FF. The management of renal angiomyolipoma. J Urol 1986;135:1121-4.
Fernández Arjona M, Mínguez R, Serrano P, Sanz J, Teba F, Peinado F, et al
. Rapidly-growing renal angiomyolipoma associated with pregnancy. Actas Urol Esp 1994;18:755-7.
Henske EP, Ao X, Short MP, Greenberg R, Neumann HP, Kwiatkowski DJ, et al
. Frequent progesterone receptor immunoreactivity in tuberous sclerosis-associated renal angiomyolipomas. Mod Pathol 1998;11:665-8.
Merran S, Vieillefond A, Peyromaure M, Dupuy C. Renal angiomyolipoma with calcification: CT-pathology correlation. Br J Radiol 2004;77:782-3.
Maizlin ZV, Gottlieb P, Corat-Simon Y, Strauss S. Various appearances of multiple angiomyolipomas in the same kidney in a patient without tuberous sclerosis. J Ultrasound Med 2002;21:211-3.
Bakshi SS, Vishal K, Kalia V, Gill JS. Aggressive renal angiomyolipoma extending into the renal vein and inferior vena cava-an uncommon entity. Br J Radiol 2011;84:e166-8.
Metro MJ, Ramchandani P, Banner MP, Siegelman ES, Stolpen AH, Wein AJ, et al.
Angiomyolipoma of the renal sinus: Diagnosis by percutaneous biopsy. Urology 2000;55:286.
Kyo CK, Won TK, Won SH, Jin SL, Hee JJ, Young DC. Trends of presentation and clinical outcome of treated renal angiomyolipoma. Yonsei Med J 2010;51:728-34.
Boorjian SA, Frank I, Inman B, Lohse CM, Cheville JC, Leibovich BC, et al
. The role of partial nephrectomy for the management of sporadic renal angiomyolipoma. Urology 2007;70:1064-8.