Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumina of vessels. The patient usually presents with nonspecific symptoms and a remarkable deterioration in performance status. The occurrence of synchronous IVLBCL and renal cell carcinoma (RCC) is extremely rare. A right kidney tumor was found in a 72-year-old man with a history of low back pain. The kidney was enlarged, with a tumor mass measuring 4.5 × 4 × 4 cm. Sections exhibited a RCC (clear cell type, nuclear grade I). Also an extensive tumor affecting capillaries and small veins was present, positive for CD45, CD20, BCL-2, and MUM1/IRF-4, consistent with IVLBCL. The lymphoma was circumscribed to the RCC. The final diagnosis was IVLBCL with a RCC as collision tumor. After that, with neurological findings, central nervous system compromise by lymphoma was made. The patient started a first cycle of chemotherapy, progressive deterioration of the sensorium, and positive blood cultures for Klebsiella pneumoniae and Escherichia coli. The patient died 8 days later of acute respiratory failure. No autopsy was done. IVLBCL is an aggressive and systemic disease characterized by massive proliferation of tumor cells without a known primary site. Clinical identification and histopathologic diagnosis are relevant issues in the therapeutic management of these lymphomas. Until now, only one case of IVLBCL coexisting with RCC has been reported. In this article, we report a second case of IVLBCL with RCC simultaneous, as an unusual collision tumor.
Keywords: B-cell lymphoma; central nervous system lymphoma; collision tumor; intravascular lymphoma; renal cell carcinoma.