The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
권호 표지

Concurrent renal and hepatic angiomyolipoma with pulmonary involvement in two patients with tuberous sclerosis

결절성경화증 환자에서 신장과 간 혈관근지방종 및 폐 침범 2예

  • Sung, Ji-Yoon (Department of Internal Medicine, Gachon University Gil Hospital, Gachon University of Medicine and Science) ;
  • Na, Sun-Young (Department of Internal Medicine, Gachon University Gil Hospital, Gachon University of Medicine and Science) ;
  • Jung, Ji-Young (Department of Internal Medicine, Gachon University Gil Hospital, Gachon University of Medicine and Science) ;
  • Lee, Hyun-Hee (Department of Internal Medicine, Gachon University Gil Hospital, Gachon University of Medicine and Science) ;
  • Chung, Woo-Kyung (Department of Internal Medicine, Gachon University Gil Hospital, Gachon University of Medicine and Science) ;
  • Sung, Yon-Mi (Department of Radiology, Gachon University Gil Hospital, Gachon University of Medicine and Science) ;
  • Chang, Jae-Hyun (Department of Internal Medicine, Gachon University Gil Hospital, Gachon University of Medicine and Science)
  • 성지윤 (가천의과대학교 길병원 내과) ;
  • 나선영 (가천의과대학교 길병원 내과) ;
  • 정지용 (가천의과대학교 길병원 내과) ;
  • 이현희 (가천의과대학교 길병원 내과) ;
  • 정우경 (가천의과대학교 길병원 내과) ;
  • 성연미 (가천의과대학교 길병원 영상의학과) ;
  • 장제현 (가천의과대학교 길병원 내과)
  • Received : 2009.10.27
  • Accepted : 2009.12.07
  • Published : 2010.12.01
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Abstract

Tuberous sclerosis (TS) involves multiple organs. Angiomyolipoma of the liver or kidney is one of the clinical manifestations of TS. However, coexistent renal and hepatic angiomyolipoma associated with TS is a rare condition. Pulmonary involvement is extremely rare, and occurs in only 0.1~1% of TS. We report two cases of concurrent renal and hepatic angiomyolipomas with pulmonary involvement in patients with TS. The first case was a 35 year-old woman who showed multiple angiomyolipomas in the liver and both kidneys and cystic parenchymal changes in the lungs. The other case was a 27-year-old woman who showed multiple angiomyolipomas in the liver and both kidneys, and multinodular pulmonary shadows. To the best of our knowledge, this is the first reported case of renal and hepatic angiomyolipomas associated with pulmonary involvement in Korea.

결절성경화증은 지능저하, 간질, 피지선종이 특징적으로 나타나는 상염색체 우성 유전 질환으로 뇌, 눈, 심장, 폐, 간, 콩팥, 피부 등 여러 장기에 양성 종양성 병변을 일으키는 질환이다. 혈관근지방종은 혈관, 평활근, 지방조직으로 구성된 양성 종양으로, 결절성경화증과 연관되어 발생하는 경우도 많다. 혈관근지방종은 신 실질에 가장 흔하며, 이외 간, 비장, 자궁관, 림프조직 등의 복부 장기 및 폐, 심장에서 발생하기도 한다. 결절성경화증에서 종양이 여러 장기에 동시에 생긴 예는 매우 드물다. 저자들은 결절성경화증 환자에서 신장과 간에 혈관근지방종이 있고 임파관평활근종증 혹은 다발성 미세결절폐세포증식증 등의 폐 침범을 나타내는 2예를 경험하였기에 보고하는 바이다.

Keywords

References

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