CTICasesLymphangioleiomyomatosis
Imaging findings
Chest radiograph and CT in a 30-year-old woman show a massive left-sided chylothorax and cystic retroperitoneal lymphatic collections in the upper abdomen. A follow-up CT scan performed more than a decade later demonstrates diffuse, thin-walled pulmonary cysts of varying sizes distributed uniformly throughout both lungs, classic for lymphangioleiomyomatosis (LAM).
Key takeaways
Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease affecting almost exclusively females. It is characterized by neoplastic proliferation of atypical smooth muscle-like cells (LAM cells) that express HMB-45. Early manifestations can include chylous pleural effusions (chylothorax) and abdominal lymphangioleiomyomas (cystic lymphatic masses) prior to the development of diffuse pulmonary cysts.
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