Metastatic hepatic leiomyosarcoma is a rare malignant smooth muscle tumor. We report a case of metastatic hepatic leiomyosarcoma associated with smooth muscle tumor of uncertain malignant potential (STUMP). A 68-year-old female presented with a liver mass (60 mm × 40 mm, Segment 4). She underwent left salpingo-oophorectomy for an ovary tumor with STUMP in a broad ligament 6 years ago. Though FDG-PET showed obvious metabolically active foci, abnormal metabolically active foci other than the lesion were not detected. A malignant liver tumor was strongly suspected and laparoscopic partial liver resection was performed with vessel-sealing devices using the crush clamping method and Pringle maneuver. Immunohistochemical findings revealed metastatic liver leiomyosarcoma associated with STUMP in a broad ligament. This case is an extremely rare case of malignant transformation from primary STUMP to metastatic hepatic leiomyosarcoma. It provides important evidence regarding the treatment for metastatic hepatic leiomyosarcoma associated with STUMP.

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