Introduction: The leiomyomatous type of uterine sarcoma with osteoclast-type giant cell component is a rare variant of uterine tumors with poor prognosis. The histological diagnosis of these rare tumors can be problematic and only five such tumors have been published previously.
Case report: A 54-year-old woman presented with fever and weight loss for 7 months and laboratory findings suggestive of inflammation. After extensive clinical investigation, a uterine tumor was found, which was considered to be an incidental finding and was thought to be unlikely to explain the symptoms. After hysterectomy, the patient had a surprising and quick recovery with the complete relief of systemic symptoms and normalization of laboratory changes. The tumor was a dedifferentiated leiomyosarcoma with osteoclast-like giant cells and contained extensive necrosis. The patient continues to do well and is tumor-free 1 year after the operation.
Discussion: To our knowledge, this is the first report of a patient being alive and disease-free 12 months after surgery with a dedifferentiated uterine leiomyosarcoma with osteoclast-like giant cells.