Retroperitoneal Lymphangioleiomyomatosis Sonography, Computed Tomography, Magnetic Resonance Imaging, and Positron Emission Tomography With Pathologic Correlation

Lymphangioleiomyomatosis (lymphangiomyomatosis [LAMB is a rare disease of unknown etiology, which almost exclusively affects women of child-bearing age. The disease is characterized histologically by diffuse hamartomatous proliferation of atypical smooth muscle cells in the lung, lymphatic trunk, and lymph nodes and causes gradual obstruction of small airways, lymphatic vessels, and vasculature.(1) The primary target of LAM is the chest, and most previous reports of LAM have concentrated on the pulmonary findings. However, extrapulmonary LAM rarely occurs with or without subsequent involvement of the lung, and several cases of extrapulmonary LAM involving abdominal or pelvic retroperitoneal organs have been reported in the radiology literature.(2-7) We describe a case of histologically proven retroperitoneal LAM in a female patient with sonography, computed tomography (CT), gadolinium-enhanced magnetic resonance imaging (MRI), and positron emission tomography (PET).