SCImago Journal & Country Rank
This journal is a member of, and subscribes to the principles of, the Committee on Publication Ethics (COPE)
2014, Volume 30, Number 3, Page(s) 233-236     
[ Abstract (Turkish) ] [ PDF ] [ Similar Articles ]
DOI: 10.5146/tjpath.2013.01190
Pulmonary Lymphangioleiomyomatosis: A Rare Case
Yetkin AĞAÇKIRAN1, Arzu ERTÜRK2, Fatma İrem YEŞİLLER2, Nevin Taci HOCA2, Leyla Nesrin ÜSTÜN3, Nermin ÇAPAN2
1Department of Pathology, Atatürk Chest Diseases and Chest Surgery Training and Research Hospital , ANKARA, TURKEY
2Department of Chest Diseases, Atatürk Chest Diseases and Chest Surgery Training and Research Hospital , ANKARA, TURKEY
3Department of Chest Surgery, Atatürk Chest Diseases and Chest Surgery Training and Research Hospital , ANKARA, TURKEY
Keywords: Lymphangioleiomyomatosis, Immunohistochemistry, Lung neoplasms, Differential diagnosis

Lymphangioleiomyomatosis is an uncommon lung disease primarily affecting women of childbearing age. It is characterized by the progressive proliferation and infiltration of smooth muscle-like cells, which lead to cystic destruction of the lung parenchyma; obstruction of airways, blood vessels, and lymphatics; and loss of pulmonary function. We present the case of a 46-year-old female patient with chest pain, cough, sputum, and dyspnea on exertion for three weeks. Minimal pneumothorax was noted, and the patient was referred to our center for further investigation and treatment. High-resolution computed tomography revealed numerous bilateral thin-walled air cysts and interstitial thickening affecting the central and peripheral part of the upper zone of the lung. We performed an open-lung biopsy to confirm lymphangioleiomyomatosis. Our aim is to discuss the pathogenesis and other lesions noted in the differential diagnosis of this rare disease.

[ Abstarct (Turkish) ] [ PDF ] [ Similar Articles ]