A Case of Pulmonary Lymphangiomyomatosis Diagnosed by Biopsy of Retroperitoneal Tumor and Treated with Hormonal Therapy

: A 34-year-old woman was admitted to our hospital because of exertional dyspnea and ascites. Chest roentgenogram showed hyperinflation of the lung and diffuse reticular shadows. Abdominal CT showed retroperitoneal tumors. Ascites was considered to be chylous. These findings strongly suggested the diagnosis of pulmonary lymphangiomyomatosis. Under lumbar anesthesia, laparotomy was performed, and the histological finding of the retroperitoneal tumor revealed angiomyoma. Bilateral oophorectomy was performed. Medroxyprogesterone at 15 mg/day was administered postoperatively. In advanced cases of this disease, open lung biopsy or even transbronchial lung biopsy is often avoided because of severe cardiopulmonary dysfunction. However, such cases associated with retroperitoneal tumor, laparotomy is quite useful not only in making the diagnosis but also in the treatment of this disease.