A 73-year-old male presented with diffuse mixed B cell lymphoma with involvement of the central nervous system (CNS) and testis manifesting as mild disorientation and aphasia. A left frontal cerebral mass and a right testicular tumor were found, and both lesions were surgically resected. Histological examination revealed diffuse mixed B cell type malignant lymphoma in the CNS and testis. The patient received irradiation to the head, and his initial symptoms improved. Pelvic computed tomography revealed enlargement of the contralateral testis and prostate. Needle biopsy confirmed lymphoma. The patient died 5 months after the initial diagnosis of septic shock. Autopsy examination revealed lymphoma cell invasion of the lung, bone marrow, prostate gland, and thalamus, but without involvement of the systemic lymph nodes. In a patient with an intracranial lymphoma, it is important to determine if the lesion is primary or metastatic and to plan medical treatment including systemic chemotherapy as soon as possible. Improvement of the prognosis of systemic non-Hodgkin's lymphoma with CNS involvement requires the detection and effective treatment of systemic lesions as well as the control of the CNS lesions.

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