Tuberc Respir Dis TRD Tuberculosis and Respiratory Diseases 1738-3536 The Korean Academy of Tuberculosis and Respiratory Diseases 10.4046/trd.2007.63.3.289 Case Report A Case of Lymphangioleiomyomatosis Presenting as a Lung Mass Park Seon Cheol M.D. 1 Park Byung Hoon M.D. 1 Shin Sang Yun M.D. 1 Jeon Han Ho M.D. 1 Chung Kyung Soo M.D. 1 Park Jun Chul M.D. 1 Jeong Jaeheon M.D. 1 Kwon Ji Eun M.D. 2 Park Moo Suk M.D. 1 3 Kim Se Kyu M.D. 1 3 Chang Joon M.D. 1 3 Kim Sung Kyu M.D. 1 3 Kim Young Sam M.D. 1 3 Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea. Department of Pathology, Yonsei University College of Medicine, Seoul, Korea. Department of Institute of Chest Diseases, Yonsei University College of Medicine, Seoul, Korea. Address for correspondence: Young Sam Kim, M.D. Department of Internal Medicine, Yonsei University, 250 Seongsanno, Seodaemun-gu, Seoul, Korea. Phone: 82-2-2228-1965, Fax: 82-2-393-6884, ysamkim@yumc.yonsei.ac.kr 09 2007 30 09 2007 63 3 289 293 09 07 2007 14 08 2007 Copyright © 2007 The Korean Academy of Tuberculosis and Respiratory Diseases 2007

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 × 3 cm 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.

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Chest X-ray shows a lung mass in right lower lung field and pneumothorax in left lung.

Chest computed tomography shows a 5 × 3 cm-sized mass in right lower lobe and multiple thin-walled small cysts scattered in both lungs.

Abdominal pelvic computed tomography shows multiple fat containing masses in both kidneys.

Biopsy of the right lower lobe mass shows proliferation of atypical smooth muscle cells and cystic dilatation of terminal bronchioles (H&E, × 100).

Immunohistochemical stain for HMB-45 shows cytoplasmic staining of smooth muscle cells.

Chest computed tomography after treatment of medroxyprogesterone. The size of the mass in right lower lobe has decreased.