{"id":"3cc309cc-d234-43df-87e3-7b23252f182b","slug":"double-hit-and-double-expressor-primary-central-nervous-system-lymphoma-experience-from-north-india-of-an-infrequent-but-aggressive-variant","title":"Double-hit and double-expressor primary central nervous system lymphoma: Experience from North India of an infrequent but aggressive variant","authors":["Mayur Parkhi","Debajyoti Chatterjee","Bishan Dass Radotra","Amanjit Bal","Budhi Singh Yadav","Manjul Tripathi"],"abstract":"Background: High-grade non-Hodgkin B-cell lymphoma is an aggressive mature B-cell lymphoma that depicts poor treatment response and worse prognosis. The presence of MYC and B-cell lymphoma 2 (BCL2) and/or B-cell lymphoma 6 (BCL6) rearrangements qualifies for triple-hit and double-hit lymphomas (THL/DHL), respectively. We attempted to explore the incidence, distribution, and clinical characteristics of the primary high-grade B-cell lymphoma of the central nervous system (CNS) in our cohort from North India. Methods: All the histologically confirmed cases of primary CNS diffuse large B-cell lymphoma (PCNS-DLBCL) over a period of 8 years were included. Cases showing MYC and BCL2 and/or BCL6 expression on immunohistochemistry (IHC) (double- or triple-expressor) were further analyzed by fluorescence in situ hybridization for MYC, BCL2 and /or BCL6 rearrangements. The results were correlated with other clinical and pathological parameters, and outcome. Results: Of total 117 cases of PCNS-DLBCL, there were seven (5.9%) cases of double/triple-expressor lymphomas (DEL/TEL) (six double- and one triple-expressor) with median age of 51 years (age range: 31–77 years) and slight female predilection. All were located supratentorially and were of non-geminal center B-cell phenotype. Only triple-expressor case (MYC+/BCL2+/BCL6+) demonstrated concurrent rearrangements for MYC and BCL6 genes indicating DHL (n = 1, 0.85%), while none of the double-expressors (n = 6) showed MYC, BCL2, or BCL6 rearrangements. The mean overall survival of the DEL/TEL was 48.2 days. Conclusion: DEL/TEL and DHL are uncommon in CNS; mostly located supratentorially and are associated with poor outcome. MYC, BCL2, and BCL6 IHC can be used as an effective screening strategy for ruling out double/ triple-expressor PCNS-DLBCLs.","thumbnailUrl":"https://sni-digital-videos.s3.amazonaws.com/articles/3cc309cc-d234-43df-87e3-7b23252f182b/featured/hero-1781560315945.png","publishDate":"2023-05-12T00:00:00.000Z","doi":"10.25259/SNI_307_2023","categories":["Neuropathology","Original Article"],"fullTextUrl":"https://surgicalneurologyint.com/api/articles/3cc309cc-d234-43df-87e3-7b23252f182b/pdf"}