Typical pulmonary lymphangioleiomyomatosis and vascular renal angiomyolipoma which was subsequently embolized to prevent hemorrhagic complications.

Left Video-Assisted Thoracoscopic Surgery (VATS)  pleurodesis prevented further pneumothoraces. A lung bulla was resected at the same time and the pathology was diagnostic of pulmonary lymphangioleiomyomatosis making her eligible for everolimus treatment. 

Sporadic lymphangioleiomyomatosis can be considered a forme fruste of tuberous sclerosis complex manifesting primarily as cystic lung disease. HMB45 is positive in both conditions (and also in melanoma).