Lymphangiomyomatosis: report of a case with ureteral involvement and chyluria

A 54-year-old woman, presenting with chyluria and progressive dyspnea, is reported. Necropsy revealed co-existent lymphangiomyomatosis and pneumoconiosis in the lungs. Retroperitoneal lymphangiomyomas surrounded the distal ureters, and cystoscopy revealed pyelolymphatic backflow into renal lymphatic sinuses. The finding of chyluria with atypical smooth muscle hyperplasia within the ureters is extremely rare. Previously reported cases of lymphangiomyomatosis are discussed and compared with our own. Electron micrographs of involved lymph nodes are presented and the findings briefly evaluated.