Role of thoracic imaging in the management of lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare diffuse lung cystic disease (DLCD) that occurs sporadically or in association with Tuberous Sclerosis Complex (TSC). The diagnostic pathway is tracked on the identification of the disease hallmarks on chest High-Resolution Computed Tomography (HRCT). Aim of this review is to discuss the thoracic HRCT pathognomonic features, essential to rule out other DLCD. It also examines the new evidences emerging from Computed Tomography (CT) quantitative studies that, by demonstrating a specific cysts distribution and a pathological aspect of the parenchyma near the cysts, could improve our understanding of this rare disorder and supply pulmonologists with a new tool for a more appropriate long-term management. Finally, the contribution of other image techniques as low dose chest CT, Magnetic Resonance Imaging (MRI) and Ultrasound (US) is discussed.