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CASE REPORT
Year : 2020  |  Volume : 47  |  Issue : 2  |  Page : 126-129

Giant renal angiomyolipoma managed by selective renal angioembolization: A unique case report


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 Submission19-Apr-2020
Date of Acceptance19-Jun-2020
Date of Web Publication11-Sep-2020

Correspondence Address:
Dr. G Ajay Kumar
Department of Urology, Sentini Hospitals, Vijayawada, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jss.JSS_27_20

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  Abstract 


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 Top


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.[1] 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.[2]

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 [3],[4],[5],[6] The management of giant AMLs is a urological challenge. Different approaches were used for management of such lesions including conservative,[7] nephrectomy,[8],[9],[10],[11] and nephron-sparing surgery (NSS).[12],[13] Reports of angioembolization in the management of giant AML are lacking. Few reports of embolotherapy included some patients with giant AMLs,[5],[6],[14],[15] and only one study was reported including only cases of giant AMLs.[16]

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.[17] Although embolization is effective for this purpose, some authors report a significant percentage of recurrent hemorrhage, recurrent symptoms, or inadequate tumor shrinkage after embolization.[18]


  Case Report Top


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 Top


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.[19] 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.[20] The second phenotype, associated with TS, usually presents in the third decade of life as multifocal bilateral renal masses.[21] The strong female prevalence suggests a hormonal component to tumor growth.[20],[22] 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.[23],[24]

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.[25],[26] 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.[27] A percutaneous renal biopsy can be helpful in dubious cases.[28]

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.[29]

NSS has been used in the management of AML. Boorjian et al.[30] 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.[12],[13] 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.[5] 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 Top


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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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