[Differential diagnosis of spontaneous pneumothorax--pulmonary lymphangioleiomyomatosis. Clinical aspects, diagnosis and therapy].

As a case report, lymphangioleiomyomatosis is presented--a rare disease that only affects women and up to now with a poor prognosis. It results from a tumorlike proliferation of smooth muscle in the thoracic and retroperitoneal lymphatics. Prominent clinical findings are dyspnea, spontaneous pneumothorax, chylous pleural effusions and chylous ascites. The diagnosis results from typical chest X-rays and is established by open lung biopsy. Treatment should primarily start with medroxyprogesteronacetat (respectively tamoxifen), if necessary with consideration of the hormonal receptors. In an early stage the oophorectomy can be helpful. Surgical intervention is necessary in complications (thoracic drainage, pleurectomy for recurrent pneumothorax, Le Veen-shunt for chylous ascites). If hormonal therapy is not successful, a combined heart and lung transplantation should be attempted in ultima ratio.