[Pulmonary lymphangiomyomatosis]

A case of pulmonary lymphangiomyomatosis in a female during menopause is presented. Difficulties in correct histopathological assessment of lung biopsies were met. The diagnosis was made after diagnosis lymph seeping into the pleural cavity. The patient was initially treated with prednisolone, tamoxifen followed by methyl progesterone and after characterizing the patient's hormone profile with danazol. Lymph seeping to the right pleural cavity was treated with pleural drainage and diet limitations. Lymph exudate to the left pleural cavity was controlled with local cytotoxic therapy. After 7 years of therapy and 8 years since the initial symptoms the state of the patient has deteriorated although she can perform every day chores and does not require oxygen therapy. High resolution CT scan disclosed extensive pulmonary changes implying extensive progression of the disease.