VEGFD and lymphangioleiomyomatosis: Indeed, although multiple, round, thin-walled cysts evenly distributed throughout the lung parenchyma at chest CT-scan are highly suggestive of LAM, its diagnosis requires at least one additional feature such as increased vascular endothelial growth factor D, the presence of renal angiomyolipoma or lymphangiomas at imaging, chylous effusion, a histopathological proof of LAM, or characteristic features of TSC, in the appropriate clinical setting [55, 56].