A Case of Lymphangioleiomyomatosis Presenting as a Lung Mass

폐종괴로 나타난 폐의 림프관평활근종증 1례

  • Park, Seon Cheol (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Park, Byung Hoon (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Shin, Sang Yun (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Jeon, Han Ho (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Chung, Kyung Soo (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Park, Jun Chul (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Jeong, Jaeheon (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Kwon, Ji Eun (Department of Pathology, Yonsei University College of Medicine) ;
  • Park, Moo Suk (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Kim, Se Kyu (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Chang, Joon (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Kim, Sung Kyu (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Kim, Young Sam (Department of Internal Medicine, Yonsei University College of Medicine)
  • 박선철 (연세대학교 의과대학 내과학교실) ;
  • 박병훈 (연세대학교 의과대학 내과학교실) ;
  • 신상윤 (연세대학교 의과대학 내과학교실) ;
  • 전한호 (연세대학교 의과대학 내과학교실) ;
  • 정경수 (연세대학교 의과대학 내과학교실) ;
  • 박준철 (연세대학교 의과대학 내과학교실) ;
  • 정재헌 (연세대학교 의과대학 내과학교실) ;
  • 권지은 (연세대학교 의과대학 병리학교실) ;
  • 박무석 (연세대학교 의과대학 내과학교실) ;
  • 김세규 (연세대학교 의과대학 내과학교실) ;
  • 장준 (연세대학교 의과대학 내과학교실) ;
  • 김성규 (연세대학교 의과대학 내과학교실) ;
  • 김영삼 (연세대학교 의과대학 내과학교실)
  • Received : 2007.07.09
  • Accepted : 2007.08.14
  • Published : 2007.09.30

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease that affects females of reproductive age. It is characterized by the abnormal proliferation of smooth muscle cells in the lung and along the axial lymphatics. We report a case of lymphangioleiomyomatosis presenting as a lung mass. The patient visited the emergency room because of dyspnea upon exertion. The chest X-ray showed a lung mass in the right lower lung field and a pneumothorax in the left lung. Chest computed tomography revealed a $5{\times}3cm$ sized mass in the right lower lobe and multiple thin-walled small cysts scattered in both lungs. Transbronchial biopsy of the lung mass was performed. The biopsy specimen showed atypical smooth muscle cell proliferation and cystic dilatation of the terminal bronchioles, which confirmed the diagnosis of lymphangioleiomyomatosis. To the best of our knowledge, this is the first case of lymphangioleiomyomatosis presenting as a lung mass.

저자들은 결절경화증과 동반하여 폐종괴로 나타난 폐의 림프관평활근종증 1예를 경험하였고 국내에서 이에 대한 보고는 없었기에 이를 문헌고찰과 함께 보고하는 바이다.

Keywords

References

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