Retroperitoneal Lymphangioleiomyomas Associated with Pulmonary Lymphangioleiomyomatosis

초록

Lymphangioleiomyomatosis (LAM) is a rare, slowly progressive disease characterized by abnormal proliferationof smooth muscle-like cells, predominantly affecting women of reproductive age. It most commonly involves the lungs, resulting in diffuse cystic destruction of the pulmonary parenchyma. Extrapulmonary involvement is rare, and presentation as a retroperitoneal mass is exceptional.We report a case of LAM presenting as a retroperitoneal mass. LAM most commonly involves the lung and predominantly affects women of reproductive age. When LAM is identified in a retroperitoneal mass, further evaluation with chest computed tomography is neccessary to assess for additional pulmonary involvement.