DISCUSSION: PEH is a rare lung tumor of vascular origin. It more commonly affects women with an average age at diagnosis of 40 years. Symptoms at presentation are often minimal, making diagnosis challenging. Imaging most commonly reveals multiple bilateral pulmonary nodules, less often as a unilateral solitary nodule, and only rarely as pleural effusions. In one study, only 11% of PEH cases presented with pleural effusions, while 20.4% had distant metastases to pleura. Clinical course varies with survival ranging from 5 months to 24 years with an average 5-year survival of 60% (47-71%) and spontaneous regression reported in 3 cases. Increased mortality is noted in those with pulmonary symptoms and hemorrhagic pleural effusions. At this time, there is no standard treatment. Due to its borderline malignancy potential, watchful waiting, surgical resection for localized tumor activity, and chemotherapy for more widespread disease have all been used with variable results.