Turkish Journal of Pathology

Türk Patoloji Dergisi

Turkish Journal of Pathology

Turkish Journal of Pathology

2014, Vol 30, Num, 3     (Pages: 233-236)

Pulmonary Lymphangioleiomyomatosis: A Rare Case

Yetkin AĞAÇKIRAN 1, Arzu ERTÜRK 2, Fatma İrem YEŞİLLER 2, Nevin Taci HOCA 2, Leyla Nesrin ÜSTÜN 3, Nermin ÇAPAN 2

1 Department of Pathology, Atatürk Chest Diseases and Chest Surgery Training and Research Hospital , ANKARA, TURKEY
2 Department of Chest Diseases, Atatürk Chest Diseases and Chest Surgery Training and Research Hospital , ANKARA, TURKEY
3 Department of Chest Surgery, Atatürk Chest Diseases and Chest Surgery Training and Research Hospital , ANKARA, TURKEY

DOI: 10.5146/tjpath.2013.01190
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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.

Keywords : Lymphangioleiomyomatosis, Immunohistochemistry, Lung neoplasms, Differential diagnosis