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We report a rare case of giant retroperitoneal dedifferentiated liposarcoma with osteosarcomatous component. The tumor was removed in a 63-year-old male that not showed any symptom. The histopathological examination highlighted a first differentiated neoplastic component and a second dedifferentiated neoplastic component where prevailed areas of osteosarcoma. To our knowledge only fourteen similar cases have been reported in the literature.
