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Park, Park, Shin, Jeon, Chung, Park, Jeong, Kwon, Park, Kim, Chang, Kim, and Kim: A Case of Lymphangioleiomyomatosis Presenting as a Lung Mass
Case Report
Tuberculosis and Respiratory Diseases

Tuberculosis and Respiratory Diseases 2007; 63(3): 289-293.

Published online: 30 September 2007

DOI: https://doi.org/10.4046/trd.2007.63.3.289

A Case of Lymphangioleiomyomatosis Presenting as a Lung Mass

Seon Cheol Park, M.D.1, Byung Hoon Park, M.D.1, Sang Yun Shin, M.D.1, Han Ho Jeon, M.D.1, Kyung Soo Chung, M.D.1, Jun Chul Park, M.D.1, Jaeheon Jeong, M.D.1, Ji Eun Kwon, M.D.2, Moo Suk Park, M.D.1, 3, Se Kyu Kim, M.D.1, 3, Joon Chang, M.D.1, 3, Sung Kyu Kim, M.D.1, 3, Young Sam Kim, M.D.1, 3

1Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

2Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.

3Department 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

Received 9 July 2007       Accepted 14 August 2007

Copyright © 2007 The Korean Academy of Tuberculosis and Respiratory Diseases

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 × 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.

Keywords: Lymphangioleiomyomatosis, Lung mass

Figures and Tables

Figure 1
Chest X-ray shows a lung mass in right lower lung field and pneumothorax in left lung.
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Figure 2
Chest computed tomography shows a 5 × 3 cm-sized mass in right lower lobe and multiple thin-walled small cysts scattered in both lungs.
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Figure 3
Abdominal pelvic computed tomography shows multiple fat containing masses in both kidneys.
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Figure 4
Biopsy of the right lower lobe mass shows proliferation of atypical smooth muscle cells and cystic dilatation of terminal bronchioles (H&E, × 100).
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Figure 5
Immunohistochemical stain for HMB-45 shows cytoplasmic staining of smooth muscle cells.
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Figure 6
Chest computed tomography after treatment of medroxyprogesterone. The size of the mass in right lower lobe has decreased.
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