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On genetic analysis, all 3 cases were diagnosed with the BHD syndrome.We report a rare case of surgical treatment for lung metastasis from high-grade uterine endometrial stromal sarcoma( ESS) with deep vein thrombosis( DVT) and pulmonary embolism(PE) in a 66-yearold woman. Chest computed tomography (CT) revealed 3 nodules of 3.9 cm, 3.3 cm, 1.2 cm in diameter in the left S6, S8, S9 of the lung. About 1 month after the hysterectomy following anticoagulant treatment with unfractionated heparin/warfarin, the patient underwent left lower lob