[BCL2, BCL6, and MYC-positive intravascular large B-cell lymphoma presenting with bilateral adrenal gland lesions]

Primary adrenal lymphoma is a rare lymphoma, accounting for <0.2% of non-Hodgkin lymphoma. The leading histopathological subtype of adrenal lymphoma is diffuse large B-cell lymphoma, and intravascular large B-cell lymphoma (IVLBCL) is rare. Here, we report a case of IVLBCL occurrence as a bilateral adrenal gland tumor, which was diagnosed by CT-guided biopsy. Tumor cells were positive for CD20 and MUM-1 but not for CD10 on immunostaining, suggesting non-germinal center B-cell subtype lymphoma. In addition, the triple expression of BCL2, BCL6, and MYC was demonstrated on tumor cells. The bone marrow examination revealed the involvement of lymphoma cells but not hemophagocytosis. The chromosomal analysis revealed complex karyotypic abnormalities without a rearrangement of BCL2 or MYC using FISH analysis. Although the patient responded to R-CHOP chemotherapy, he developed central nervous system involvement by lymphoma. To date, the significance of the triple expression of BCL2, BCL6, and MYC without gene translocation remains partially elucidated. Therefore, an accumulation of similar cases is needed to elucidate the pathogenesis and clinical significance of the triple expression of these oncoproteins.