Journal List > Korean J Urol > v.48(3) > 1004885

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Kim, Lee, Kim, Oh, Bae, Park, Kim, and Shim: Spontaneous Rupture of Renal Angiomyolipoma in a Female Tuberous Sclerosis Patient with Pulmonary Lymphangioleiomyomatosis
Case Report
Korean Journal of Urology

Korean Journal of Urology 2007; 48(3): 344-347.

Published online: 31 March 2007

DOI: https://doi.org/10.4111/kju.2007.48.3.344

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

Jong Wook Kim, Tae Won Lee, Myung Joon Kim, Mi Mi Oh, Jae Hyun Bae, Hong Seok Park, Je Jong Kim, Kang Soo Shim

Department of Urology, College of Medicine, Korea University, Seoul, Korea.

Corresponding author (uroshim@korea.ac.kr)

Received 4 January 2007       Accepted 13 February 2007

Copyright © 2007 The Korean Urological Association

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.

Keywords: Angiomyolipoma, Tuberous sclerosis, Lymphangioleiomyomatosis

Figures and Tables

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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