RESUMO
A 34-year old woman underwent a surgical intervention as a result of a retroperitoneal tumor. This tumor was excised and the pathological diagnosis was lymphangiomyoma. Two months later she developed a spontaneous right pneumothorax with a chylous pleural effusion. Roentgenograms showed that the lungs had developed a honeycomb pattern. She was treated surgically and at the same time a biopsy was performed. Sixteen months later she deveoped a left pneumothorax which necessitated additional surgery, and a further biopsy was taken. She died of respiratory insufficiency 2 years after the clinical onset of the symptoms. The pathologic material was studied by both electron and light microscopy in serial sections. The electron-microscopic study proved the muscular nature of this condition. The light microscopic study of the serial sections showed that the lung lesion was due to the proliferation of the muscle cells associated with the lymphatics surrounding the acini.
