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Saudi Journal of Kidney Diseases and Transplantation
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LETTER TO THE EDITOR  
Year : 2017  |  Volume : 28  |  Issue : 3  |  Page : 675-677
Asymptomatic bilateral giant angiomyolipoma


Department of Nephrology, University of Arizona Medical Center, 1501 North Campbell Ave, Tucson, AZ, United States

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Date of Web Publication18-May-2017
 

How to cite this article:
Kaur G, Thajudeen B. Asymptomatic bilateral giant angiomyolipoma. Saudi J Kidney Dis Transpl 2017;28:675-7

How to cite this URL:
Kaur G, Thajudeen B. Asymptomatic bilateral giant angiomyolipoma. Saudi J Kidney Dis Transpl [serial online] 2017 [cited 2020 Feb 28];28:675-7. Available from: http://www.sjkdt.org/text.asp?2017/28/3/675/206449
To the Editor,

Angiomyolipoma (AML) is a benign hamar- tomatous lesion consisting of mature adipose tissue, smooth muscle, and thick-walled vessels.[1] Its prevalence is estimated at 0.3% of the population and accounts for 3% of the solid renal masses. It occurs sporadically in 80% of the cases. Twenty percent of cases are related to hereditary syndromes such as tuberous sclerosis, lymphangioleiomyomatosis, and Von Hippel-Lindau syndrome.[1] Incidental detection of giant AML has been rarely reported in literature. A 62-year-old female patient with no significant past medical history was evaluated for elevated liver enzymes noted on routine laboratory testing. She never complained of abdominal pain. On physical examination, a large mass was noted involving the right flank and iliac region, extending to the hypogastric area, and going beyond the mid- line. Ultrasound revealed bilateral massively enlarged kidneys. An magnetic resonance imaging (MRI) of the abdomen showed enlarged kidneys measuring 17 cm – 11 cm – 26 cm on the right and 12 cm – 13 cm – 21 cm on the left with extensive fatty replacement [Figure 1]. The lower pole of the kidney was extending as far as the pelvis [Figure 2] and [Figure 3]. Laboratory tests showed normal renal function, trace proteinuria, and no hematuria. Although the patient was asymptomatic, because of concern for hemorrhage in the future, surgery- or interventional radiology- assisted renal arterial embolization was suggested both of which were refused by the patient. We decided to do a strict regimen of surveillance which includes an enquiry about symptoms, physical examination, and MRI abdomen every six months.
Figure 1: Magnetic resonance imaging abdomen and pelvis coronal view showing the giant right renal angiomyolipoma and portion of left renal angiomyolipoma.

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Figure 2: Magnetic resonance imaging abdomen and pelvis coronal view showing bilateral giant renal angiomyolipoma.

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Figure 3: Magnetic resonance imaging abdomen and pelvis sagittal view showing the lower extent of angiomyolipoma.

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Sporadic AML has a female preponderance and commonly seen between fifth and sixth decades of life. The most common signs and symptoms are abdominal pain, palpable abdominal mass, and hematuria, and the symptoms are related to its size and rapidity of growth. A computed tomography (CT) scan or MRI can establish the diagnosis. Although detection of fat is the classic imaging feature, a hyper- attenuating appearance on unenhanced CT or a T2-hypointense appearance at MRI identifies smooth muscle component which is an important diagnostic sign in AML that contains few or no fat cells.

Histopathology of AML shows blood vessels, plump spindle cells, and adipose tissue. AML is generally slow growing, and in a study of 35 patients followed clinically for four years by Steiner et al, 64% had no change in the size during follow-up.[2]

A recent retrospective review of 447 patients with AML over 43 months showed that the vast majority were sporadic, asymptomatic (90%), and initially harmless, with a negligible growth rate (<0.02 cm/year) which supports the adoption of active surveillance for all asymptomatic AMLs.[3] But at the same time, the European Association of Urology guidelines recommend treatment in cases of large tumors, those in women of childbearing age, and where follow-up or access to emergency care is inadequate.[4] Consideration should also be given to the increased intra- and post-operative complications associated with removal of larger tumors. Nephron-sparing surgical procedure for large tumors includes tumor enucleation, selective angioembolization, and partial nephrectomy.[5] Pharmacological treatment of AML has been attempted using mammalian target of rapamycin inhibitors such as sirolimus.

Conflict of interest: None declared.

 
   References Top

1.
Jinzaki M, Silverman SG, Akita H, Nagashima Y, Mikami S, Oya M. Renal angiomyolipoma: A radiological classification and update on recent developments in diagnosis and management. Abdom Imaging 2014;39:588-604.  Back to cited text no. 1
[PUBMED]    
2.
Steiner MS, Goldman SM, Fishman EK, Marshall FF. The natural history of renal angiomyolipoma. J Urol 1993;150:1782-6.  Back to cited text no. 2
[PUBMED]    
3.
Bhatt JR, Richard PO, Kim NS, et al. Natural history of renal angiomyolipoma (AML): Most patients with large AMLs >4cm can be offered active surveillance as an initial management strategy. Eur Urol 2016;70:85-90.  Back to cited text no. 3
[PUBMED]    
4.
Kuusk T, Biancari F, Lane B, et al. Treatment of renal angiomyolipoma: Pooled analysis of individual patient data. BMC Urol 2015; 15:123.  Back to cited text no. 4
    
5.
Schneider-Monteiro ED, Lucon AM, de Figueiredo AA, Rodrigues Junior AJ, Arap S. Bilateral giant renal angiomyolipoma associated with hepatic lipoma in a patient with tuberous sclerosis. Rev Hosp Clin Fac Med Sao Paulo 2003;58:103-8.  Back to cited text no. 5
    

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Correspondence Address:
Dr. Bijin Thajudeen
Department of Nephrology, University of Arizona Medical Center, 1501 North Campbell Ave, Tucson, AZ
United States
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DOI: 10.4103/1319-2442.206449

PMID: 28540918

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  [Figure 1], [Figure 2], [Figure 3]



 

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