BACKGROUND: Liposarcoma and lymphoma are very rare tumors, and their combination is extremely rare. Moreover, there have been no reports of liposarcoma and lymphoma occurring in the same region. CASE: A 58-year-old man presented to Kanagawa Cancer Center with a mass in his left thigh and underwent a needle biopsy. Histological analysis showed an increase in the number of small lymphocytes and plasma cells
immunohistochemical analysis showed an increase in CD20-positive cells with Lambda light-chain restriction
therefore, the diagnosis of B-cell malignancy with plasma cell differentiation was made. A bone marrow biopsy specimen showed infiltration of atypical cells of the same phenotype and increased serum IgM-M levels
therefore, a diagnosis of Waldenström macroglobulinemia/lymphoplasmacytic lymphoma (LPL) was made. The needle biopsy specimen showed scattered CDK4-positive cells in the background of the lymphoma cells and sporadic MDM2 signal amplification on fluorescence in situ hybridization, suggesting mixed well-differentiated liposarcoma (WDL). Tumor resection was performed. The tumor contained a mixture of WDL and LPL areas. RNA sequencing revealed upregulated expression of chemokine genes, including CCL5, CCL18, and CCL19, in WDL and that of the corresponding chemokine receptor genes CCR4, CCR6, and CCR7 in the lymphoma cells. CONCLUSION: Chemokine-chemokine receptor axes may be involved in the pathogenesis of LPL cell-infiltrating WDL. This is an extremely rare case, and we have reported some considerations regarding the tumorigenesis of LPL cell-infiltrating WDL.