학술논문
폐종괴로 나타난 폐의 림프관평활근종증 1례
A Case of Lymphangioleiomyomatosis Presenting as a Lung Mass
A Case of Lymphangioleiomyomatosis Presenting as a Lung Mass
Document Type
Article
Author
박선철 / Seon Cheol Park; 박병훈 / Byung Hoon Park; 신상윤 / Sang Yun Shin; 전한호 / Han Ho Jeon; 정경수 / Kyung Soo Chung; 박준철 / Jun Chul Park; 정재헌 / Jae Heon Jeong; 권지은 / Ji Eun Kwon; 박무석 / Moo Suk Park; 김세규 / Se Kyu Kim; 장준 ( Joon Chang
Source
Tuberculosis and Respiratory Diseases. Sep 30, 2007 63(3):289
Subject
Language
Korean
ISSN
1738-3536
Abstract
저자들은 결절경화증과 동반하여 폐종괴로 나타난 폐의 림프관평활근종증 1예를 경험하였고 국내에서 이에 대한 보고는 없었기에 이를 문헌고찰과 함께 보고하는 바이다.
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. (Tuberc Respir Dis 2007; 63: 289-293)
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. (Tuberc Respir Dis 2007; 63: 289-293)