36. Quadruple-hit B-cell lymphoma with simultaneous BCL2, BCL6, CCND1, and MYC rearrangements: Two new cases

      The WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues has a section on high-grade B-cell lymphoma (HGBL) with MYC translocations, which includes double/triple-hit lymphomas characterized by MYC and BCL2 and/or BCL6 rearrangements, as well as other aggressive lymphomas without a MYC translocation that have a blastoid morphology or a morphology intermediate between DLBCL and BL (HGBL, NOS). 'Quadruple-hit' cases, defined by the presence of MYC, BCL2, BCL6, and CCND1 rearrangements, are rare; only three cases have been reported (
      • Bacher U
      • et al.
      Several lymphoma-specific genetic events in parallel can be found in mature B-cell neoplasms.
      ,
      • Ittel A
      • et al.
      Four genetic lymphoma-specific events (MYC, BCL2, BCL6 and CCND1) identified in a high-grade B lymphoma case.
      ). We report two new cases of HGBL characterized by MYC, BCL2, BCL6, and CCND1 rearrangements and positive cyclin D1 immunostaining. Both cases were examined by FISH and one by conventional cytogenetics. It is challenging to classify CCND1-positive lymphomas that also harbor BCL2, BCL6, and MYC rearrangements. Perhaps the best terminology might be 'HGBL with BCL2, BCL6, CCND1, and MYC rearrangements'. The challenge is not only confined to classification, but also to the best treatment options. Similar cases need to come forward to better understand the biology of this entity. We hypothesize that these lymphomas might be more common than currently recognized, but may go undetected because cyclin D1 is not always performed, and CCND1 is not typically included in the triple-hit FISH panel.
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      References

        • Bacher U
        • et al.
        Several lymphoma-specific genetic events in parallel can be found in mature B-cell neoplasms.
        Genes Chromosom Cancer. 2011; 50: 43-50
        • Ittel A
        • et al.
        Four genetic lymphoma-specific events (MYC, BCL2, BCL6 and CCND1) identified in a high-grade B lymphoma case.
        Blood Cancer J. 2015; 5: e374