2003 Volume 17 Issue 1 Pages 44-47
Pulmonary lymphangioleiomyomatosis (LAM) is an uncommon disease afflicting women of childbearing age. The disease is characterized histologically by proliferation of smooth muscle in the lung. LAM is generally diagnosed with chest computed tomography (CT) scan, video-assisted thoracoscopic surgery (VATS), autopsy, transbronchial lung biopsy (TBLB). The patient was a 36-year-old female with chest pain. She was admitted because of spontaneous pneumothorax diagnosed on X-ray. There was no characteristic lesion on chest CT, or diffuse cystic change detected after chest tube drainage. Air leakage persisted. We performed VATS because of persistent air leakage after chest drainage. VATS persisted that the presence of multiple small granular nodules and telangiectasia on the surface of the right upper lobe. Histologically, the biopsy specimen demonstrated emphysema-like lesions and smooth muscle proliferation in the lung. Smooth muscle actin (SMA) was positive. Human melanoma black (HMB)-45, estrogen receptor, and progesterone receptor were negative. We diagnosed this patient as being in the early stage of LAM. We conclude that VATS is useful for diagnosing the early stage of LAM and lung biopsy with VATS is necessary for pneumothorax in women of childbearing age.
