[An early stage case of pulmonary lymphangiomyomatosis]

A 35-year-old unmarried woman was admitted to our hospital because of right pneumothorax. Pulmonary lymphangiomyomatosis was suspected from CT and transbronchial lung biopsy findings. The diagnosis was confirmed histologically by open lung biopsy. As her biopsy specimen showed positive findings for both estrogen and progesterone receptor, tamoxifen administration was started. The CT revealed multiple cystic lesions (5-10 mm in diameter) but otherwise normal vascular structures and normal lung density. The plain chest radiograph showed no abnormalities. CT is very useful for the early detection of pulmonary lymphangiomyomatosis.