A Case of Lymphangioleiomyomatosis Presenting as a Lung Mass.

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

doi:2007.63.3.289

Tuberc Respir Dis > Volume 63(3); 2007 > Article

Case Report

Tuberculosis and Respiratory Diseases 2007;63(3):289-293.
DOI: https://doi.org/10.4046/trd.2007.63.3.289 Published online September 1, 2007.

A Case of Lymphangioleiomyomatosis Presenting as a Lung Mass.

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

¹Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea. ysamkim@yumc.yonsei.ac.kr
²Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
³Institute of Chest Diseases, Yonsei University College of Medicine, Seoul, Korea.

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 x 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.

Key Words: Lymphangioleiomyomatosis, Lung mass