Abstract
Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease that occurs primarily in women of childbearing age. In addition, pulmonary LAM is an important basal disease in the occurrence of spontaneous pneumothorax in women. We report two cases of pulmonary LAM that followed different courses. Case 1: a 26-year-old woman with a right renal tumor developed spontaneous pneumothorax. Chest CT scans revealed multiple thin-walled cystic lesions in both lung fields. A partial pulmonary excision was performed by thoracoscopic surgery and pleurodesis was performed by pleura paratripsis. The pathological diagnosis was pulmonary LAM. The right kidney tumor was removed by total extirpation of the right kidney, and a liver tumor was treated by partial hepatectomy. The pathological diagnosis of the tumor specimens was angiomyolipoma. Case 2: a 45-year-old woman was admitted to our hospital for sudden dyspnea and left chest pain. Chest CT scans revealed left spontaneous pneumothorax, with multiple thin-walled cystic lesions in both lung fields. A partial pulmonary excision was performed by thoracoscopic surgery and pleurodesis was performed by pleura paratripsis. The pathological diagnosis was pulmonary LAM. Currently, Case 1 shows a mass recurrence in the liver and lungs during one year after the partial hepatectomy, while Case 2 does not show evidence of recurrence during one year after the partial pulmonary excision. These two cases show that the clinical course of LAM varies. Therefore, for every case, an appropriate choice of regimen and performing complete follow-up examinations are important.
