Korean J Urol.  2007 Mar;48(3):344-347. 10.4111/kju.2007.48.3.344.

Spontaneous Rupture of Renal Angiomyolipoma in a Female Tuberous Sclerosis Patient with Pulmonary Lymphangioleiomyomatosis

• Kim JW
• Lee TW
• Kim MJ
• Oh MM
• Bae JH
• Park HS
• Kim JJ
• Shim KS

• KMID: 1997135
• DOI: http://doi.org/10.4111/kju.2007.48.3.344

Abstract

Pulmonary lymphangioleiomyomatosis (LAM) is uncommon debilitating disease, predominantly affecting women of childbearing age. Renal angiomyolipoma (AML) is the most common renal lesion in patients with tuberous sclerosis (TS), but, even without TS, is reported in up to 60% of the patients with LAM, which is frequently diagnosed several years before the LAM itself. However, it is extremely rare for renal AML and pulmonary LAM to simultaneously present in tuberous sclerosis complex. Herein, a case of spontaneous rupture of a renal AML, accompanied by pulmonary LAM, in a reproductive female with TS, managed by selective transcatheter arterial embolization (TAE), is reported.

Keyword

Angiomyolipoma; Tuberous sclerosis; Lymphangioleiomyomatosis

MeSH Terms

Angiomyolipoma*
Female*
Humans
Lymphangioleiomyomatosis*
Rupture, Spontaneous*
Tuberous Sclerosis*

Figure

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  Fig. 1 Reticulonodular opacities in the parenchyma of both lungs.

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  Fig. 2 Computed tomography showing a right angiomyolipoma (AML) rupture with a severe perirenal hematoma.

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  Fig. 3 Multiple cystic destruction lesions of the lung parenchyma, with a pneumothorax.

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  Fig. 4 (A) Right angiography showing various multiple aneurysms. (B) After transcatheter arterial embolization (TAE), the aneurysms and leakage have disappeared (arrow).

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  Fig. 5 (A) Multiple iso-signal nodular lesions near the lateral ventricle in the T1WI (arrowhead). (B) Multiple low-signal nodular lesions near the lateral ventricle in the T2WI (arrowhead).

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