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| Year : 2012 | Volume
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| Issue : 2 | Page : 358-360 |
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| Renal angiomyolipoma |
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SM Badruddoza, RA Jahan
Department of Pathology, Rajshahi Medical College, Rajshahi, Bangladesh
Click here for correspondence address and email
| Date of Web Publication | 28-Feb-2012 |
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How to cite this article:
Badruddoza S M, Jahan R A. Renal angiomyolipoma. Saudi J Kidney Dis Transpl 2012;23:358-60 |
To the Editor,
Renal angiomyolipoma is an uncommon benign mesenchymal tumor of the kidney. It contains fat, smooth muscle, and tortuous blood vessels in varying proportions. First described by Bourneville in 1880, [1] it is often considered to be a hamartoma rather than a malignant neoplasm. Two forms of this tumor are recognized clinically; the first is associated with tuberous sclerosis, where the tumor is usually small, multiple, bilateral and has an equal sex distribution. The second form occurs more often in women and is not associated with tuberous sclerosis, and the tumor is often large, single, and unilateral. [2]
A 46-year-old woman presented to her general practitioner with a two month history of right-sided flank pain, hematuria, and weight loss. She was not diabetic or dyslipidemic and there was no significant past medical or family history. She did not take any medications or had a history of illicit intravenous drug usage.
Physical examination revealed a healthy woman. There was no pallor and her blood pressure was normal. The serum biochemical profile was normal. Routine examination of urine revealed hematuria. Ultrasonography revealed a mass originating from the lower pole of the right kidney.
A preoperative diagnosis of a large solitary right renal tumor was made. At operation, an irregularly shaped tumor consisting mainly of fat was found adherent to the inferior pole of the right kidney. The right ureter was stretched over the upper surface of this tumor. The mass measuring about 8.0 × 5.5 × 5.0 cm and weighing 200 g was excised together with a small rim of normal renal tissue [Figure 1].
Histopathology confirmed the diagnosis of a renal angiomyolipoma with the characteristic picture of a tumor composed of mature fat cells, irregular, thick-walled blood vessels, and bundles of smooth muscle cells [Figure 2]. | Figure 2: Renal angiomyolipoma showing mature fat cells, blood vessels, and smooth muscle cells (H&E ×400).
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Postoperative recovery was uneventful and the patient was discharged in the eighth postoperative day.
Renal angiomyolipoma arises from the me-senchymal elements of the kidney. [3] These tumors consist of a collection of blood vessels, smooth muscle, and mature adipose tissue, although a more aggressive subtype also exists that consists of perivascular epithelioid cells. [4] A total of 80% of angiomyolipoma are associated with tuberous sclerosis. [5] A sporadic and rare form of angiomyolipoma is also found where lesions are usually single, unilateral, and generally asymptomatic. [6] Renal angiomyolipoma are commonly found as incidental findings on cross-sectional imaging (ultrasound, computed tomography or magnetic resonanceimaging). [7] On ultrasound, they usually cast an acoustic shadow and appear homogeneous with high reflectivity due to their high fat content. [5] On CT, angiomyo-lipoma have well-defined margins, with a variable proportion of fat and soft tissue, although the former usually predominates. [4] Histology of angiomyolipoma confirms that they are mainly composed of spindle-shaped smooth muscle cells, and the presence of multiple epithelioid cells is found in the more aggressive form. [8] The majority of patients with renal angiomyolipoma are asymptomatic and it is estimated that over ten million people worldwide have such lesions. [7] In the minority of patients that are symptomatic, the classic "Lenk's triad" of symptoms include flank or abdominal pain, a palpable or tender mass and hematuria [9] Other symptoms may include fever,
vomiting, anemia, renal failure, and hypo-tension. [4] Some angiomyolipoma can grow rapidly, up to 4 cm per year. [10] If greater than 4 cm in diameter, these lesions are associated with an increased risk of aneurysm formation and hence a higher possibility of rupture and hemorrhage. [9] If bleeding presents acutely, it is potentially a life-threatening situation, which may require surgical intervention leading to a possible nephrectomy [11] In all cases, the aim of treatment is to preserve renal function and to prevent hemorrhage. [7]
It is important to recognize the benign nature of these tumors and their characteristic ultra-sonography and CT findings. So that diagnosis of an aggressive neoplasm particularly renal cell carcinoma can be prevented. Most of these tumors are amenable to conservative management with frequent follow-up. Even when surgical excision is indicated, the kidney itself can often be spared without jeopardizing the patient. Nephrectomy is necessary only when the tumor has replaced the whole kidney or when there is a concomitant renal carcinoma present.
 References | |  |
| 1. | Bourneville DM. Contribution to the study of idotic, circonovolutions tuberous sclerosis of the brain: idiocy and epilepsy hemipleque. Arch Neurol 1880;1:84. 
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| 2. | Yip FW, Lee SH. Renal angiolipomata- A case report. Singapore Med J 1992;33:643-4.
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| 3. | Simmons JL, Hussain SA, Riley P, Wallace DM. Management of renal angiomyolipoma in patients with tuberous sclerosis complex. Oncol Rep 2003;10:237-41.
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| 4. | Unlu C, Lamme B, Nass P, Bolhuis HW. Retroperitoneal haemorrhage caused by a renal angiomyolipoma. Emerg Med J 2006;23:464-5.
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| 5. | Lewis EL, Palmer JM. Renal angiomyolipoma and massive retroperitoneal haemorrhage during pregnancy. Western J Med 1985;143:675-6.
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| 6. | Yamakado K, Tanaka N, Nakagawa T. Renal angiomyolipoma: relationships between tumour size, aneurysm formation, and rupture. Radiology 2002;225:78-82.
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| 7. | Wagner BJ, Wong-You-Cheong JJ, Davis CJ. Adult renal hamartomas. Radiographics 1997; 1:155-69.
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| 8. | Bernardini S, Chabannes E, Algros M, Billerey C, Bittard H. Variants of renal angiomyolipoma: Difficulties in the histological diagnosis. Urol Int 2002;69 : 78-81.
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| 9. | Kunzi T, Walther F, Marti HP, Frey FJ, Vogt B. Intrarenal arterial aneurysms with haematuria in a patient with tuberous sclerosis complex. Nephrol Dialysis Transpl 2005;20:2268-70.
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| 10. | Steiner MS, Goldman SM, Fishman EK, Marshall FF. The natural history of renal angiomyolipoma. J Urol 1993;150:1782-6.
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| 11. | Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyoli-poma. J Urol 2002;168:1315-25.
[PUBMED] |
Correspondence Address:
S M Badruddoza
Department of Pathology, Rajshahi Medical College, Rajshahi
Bangladesh
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PMID: 22382238 
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