Autopsy Findings of Retroperitoneal Cystic Tumor and Peliosis Hepatis in Lymphangiomyomatosis

: A 40-year-old woman who worked as a nurse and had suffered from progressive exertional dyspnea for about 14 years underwent open lung biopsy with surgical treatment for pneumothorax. The diagnosis was lymphangiomyomatosis and she was treated with danazol to suppress ovarian function. Her condition improved temporarily, but she died of respiratory failure when she was 47 years old. The survival time after the onset of respiratory symptoms was 21 years, and after the biopsy it was 8 years. At autopsy a retroperitoneal cystic tumor was found (9 x 7 x 5 cm), which had been evident clinically. Histologic examination showed that the tumor was an extrapulmonary manifestation of the lumphangiomyomatosis lesion. Some paraaortic lymph nodes has similar lesions. Aggregates of small red spots were seen on acute surface of the liver. These were diagnosed as peliosis hepatis, they may have been caused by the danazol.