Malignant lymphoma sometimes originates from extranodal sites; however, the uterus has rarely been reported as the site of the primary lesion. We present a patient with malignant lymphoma of the uterus complicating bilateral hydronephrosis. A 67-year-old previously healthy woman was seen at a clinic because of massive genital bleeding. She was referred to our hospital for further examination of a uterine tumor. Computed tomography scans revealed a pelvic tumor invading to the retroperitoneal region, which caused bilateral obstruction of the ureters and hydronephrosis. No lymph node swelling was detected. Magnetic resonance imaging showed a bulky uterine tumor that was homogenously low on T1-weighted imaging and isointense on T2-weighted imaging, while the endometrium was intact. A pathological examination of the biopsy specimen from the uterine cervix revealed diffuse infiltration of CD20-positive atypical large lymphoid cells, which was compatible with diffuse large B-cell lymphoma (DLBCL). Since the tumor expanded from the uterus and no other abnormal lesion was observed in imaging studies including gallium scintigraphy, a diagnosis of DLBCL of the uterus, clinical stage IE was made. The patient received six cycles of rituximab plus CHOP chemotherapy followed by involved field irradiation. She achieved complete remission and has been alive for more than two years without relapse.

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