Primary renal lymphoma is a rare entity representing less than 1% of lesions in the kidney. We present the case of a 42-year-old male who was evaluated for pain and a mass in the abdomen. The computed tomogram of the abdomen showed a large lobulated homogeneously enhancing mass lesion of about 14×12×18 cm, involving the whole of the left kidney and encasing the left renal vessels and ureter. The patient underwent a biopsy, and the histopathology was diffuse large B cell lymphoma, positive for LCA, CD20, PAX 5, Bcl 2 and negative for SIgM, CD33, CD34, CD5, Tdt, with MIB 1 labelling index of 40%. He received chemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (R CHOP) for eight cycles followed by radiation to the residual mass and achieved complete remission. Currently, he is alive and in remission at 28 months.
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