Presentation
Abdominal discomfort worse before menstruation.
Patient Data
water-attenuation mass extending along the right iliac vessels and insinuating between and enveloping normal structures without mass-effect
few small lung cysts
no effusions
no lymphadenopathy
no AMLs
innumerable scattered thin-walled small lung cysts
normal intervening lung parenchyma
right pneumothorax
scattered lung cysts which have increased in size. and number
hyperattenuating bilateral pleural thickening due to prior VATS pleurodesis
left pneumothorax
Case Discussion
Diagnosis of sporadic lymphangioleiomyomatosis (s-LAM) confirmed.
Abdominal lymphangioleiomyoma was found to vary in size with her menstrual cycle but reduced in size following initiation of sirolimus therapy.
Lymphatic abnormalities are a feature of s-LAM.