An entity that poses continuous challenge despite treatment attempt: lymphangioleiomyomatosis

摘要

In May of 2016, a 37-year-old Hispanic female, withno significant past medical history was brought toour hospital with right-sided chest pain. Chest X-rayrevealed large right pneumothorax (A) and sheunderwent chest tube placement. Computedtomography (CT) chest without contrast showedinnumerable cysts in the upper and lower lobes ofbilateral lungs (B) and right thoracostomy tube withvery small residual pneumothorax (C). CT abdomenshowed hyperdense mass in left and possibly rightkidney (D). CT head without contrastshowed small calcifications in the lateralventricles suggestive of tubers (E). A diagnosis of lymphangioleiomyomatosis (LAM) was suspected.Serum vascular endothelial growth factor D was 800 pg/ml. She underwent open lung biopsy anddiagnosis of LAM was established afterhistopathologic evaluation. Patient was sent toLAM clinic at a higher center and was started onsirolimus with an aim to stabilize her lung function,improve quality of life and functional performance.