We present a rare case of dedifferentiated liposarcoma confined to the spleen and splenic hilum. An 81-year-old man was referred to our hospital with a large asymptomatic splenic tumor. The patient underwent splenectomy, and the adipose tissue surrounding the splenic hilum was also resected. Microscopically, the tumor mainly consisted of high-grade spindle cells similar to those seen in undifferentiated pleomorphic liposarcoma. In the splenic hilum, scattered atypical cells were detected in the sclerosing component and adipose tissue. Immunohistochemically, both the spindle cells in the spleen and the atypical cells in the splenic hilum were positive for MDM2 and CDK4. The histopathologic diagnosis was dedifferentiated liposarcoma derived from an atypical lipomatous tumor/well-differentiated liposarcoma of the adipose tissue in the splenic hilum with extension into the spleen. Dedifferentiated liposarcoma in the spleen and splenic hilum should be considered as a differential diagnosis of splenic tumors.
Keywords: Chondro-osseous differentiation; Dedifferentiated liposarcoma; Retroperitoneal tumor; Spleen.