Pulmonary lymphangioleiomyomatosis associated with a fatal pneumothorax: an autopsy case report.

Korean J Leg Med. 2000 Oct;24(2):61-65. Korean.

Department of Forensic Pathology, National Institute of Scientific Investigation, Seoul, 158-097, Korea.

Pulmonary lymphangioleiomyomatosis is a rare disease which is defined as an abnormal proliferation of smooth muscles around lymphatics, venules, and bronchioles. The most frequent presenting complaint is dyspnea, either in conjunction with pneumothorax, chylothorax, or on exertion. Common extrapulmonary features are retroperitoneal adenopathy, tuberous sclerosis and renal angiomyolipomas. This case was a 34-year-old pregnant woman who died of pneumothorax. She had no past history of illness. At autopsy, both lungs showed honey-comb appearance, and was proven histopathologically pulmonary lymphangioleiomyomatosis. The proliferating smooth muscles were immunoreactive for alpha-smooth muscle actin and HMB-45. Multiple angiomyolipomas of left kidney coexisted. However, no stigmata of tuberous sclerosis was present. Pregnancy was thought to aggravate her clinical course.

Key words: Lymphangioleiomyomatosis, lung, pneumothorax, angiomyolipoma