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Case Reports
. 2016 May;31(3):223-6.
doi: 10.5001/omj.2016.42.

Aneurysm in a Large Sporadic Renal Angiomyolipoma

Affiliations
Case Reports

Aneurysm in a Large Sporadic Renal Angiomyolipoma

Bedoor Al Omran et al. Oman Med J. 2016 May.

Abstract

Angiomyolipomas (AMLs) are the most common mesenchymal renal neoplasms and are classified as neoplasms of perivascular epithelioid cells (PEComa). AML is usually a benign neoplasm arising most often in the kidney although it has been described in a wide variety of sites. Most patients are adults, and one-third suffer from tuberous sclerosis. We describe a case of renal AML in a 54-year-old Bahraini woman who presented to the Bahrain Defence Force Hospital with right flank pain and hematuria, and who was known to have rheumatoid arthritis but had no cutaneous or other stigmata of tuberous sclerosis. It is the largest AML reported in Bahrain and is also striking for the fact that it contained an intratumoral aneurysm that ruptured causing symptoms leading to the radiological diagnosis of renal mass. Furthermore, the occurrence of an aneurysm in sporadic AML, as in our case, is rare since the large majority tend to be seen in association with tuberous sclerosis.

Keywords: Aneurysm; Angiomyolipoma; Tuberous Sclerosis.

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Figures

Figure 1
Figure 1
Computed tomography scan showing the tumor.
Figure 2
Figure 2
Contrast computed tomography scan showing the aneurysm.
Figure 3
Figure 3
Right kidney. (a) Mass arising from the posteromedial aspect, measuring 10.5 cm, stretching the capsule but not breaching it. (b) The cut surface of the mass was fleshy and tan-red, with extensive hemorrhage.
Figure 4
Figure 4
Hematoxylin and eosin staining revealed (a) the three components of angiomyolipoma: fat, blood vessels, and smooth muscle. The blood vessels showed irregular, thickened walls and spindled smooth muscle cells, magnification = 100 ×. (b) Epithelioid myoid cells between fat and vascular structures were visible, magnification = 250 ×. (c) Immunoperoxidase staining of myoid (smooth muscle cells) was positive for smooth muscle actin, magnification = 100 ×. (d) Perivascular myoid cells showed positive staining for HMB-45, magnification = 250 ×.
Figure 5
Figure 5
Aneurysmal dilatation of blood vessel 5 mm in diameter. The red arrow shows focal disruption and lifting of the endothelium (hematoxylin and eosin stain, magnification = 250 ×).

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