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Diffuse large B-cell lymphoma of Waldeyer's ring has distinct clinicopathologic features: a GELA study

Authors :
de Leval, L.
Bonnet, C.
Copie-Bergman, C.
Seidel, L.
Baia, M.
Brière, J.
Molina, T. J.
Fabiani, B.
Petrella, T.
Bosq, J.
Gisselbrecht, C.
Siebert, R.
Tilly, H.
Haioun, C.
Fillet, G.
Gaulard, P.
de Leval, L.
Bonnet, C.
Copie-Bergman, C.
Seidel, L.
Baia, M.
Brière, J.
Molina, T. J.
Fabiani, B.
Petrella, T.
Bosq, J.
Gisselbrecht, C.
Siebert, R.
Tilly, H.
Haioun, C.
Fillet, G.
Gaulard, P.

Abstract

Background Diffuse large B-cell lymphomas (DLBCLs) arising in specific extranodal sites have peculiar clinicopathologic features. Patients and methods We analyzed a cohort of 187 primary Waldeyer's ring (WR) DLBCLs retrieved from GELA protocols using anthracyclin-based polychemotherapy. Results Most patients (92%) had stage I-II disease. A germinal center B-cell-like (GCB) immunophenotype was observed in 61%, and BCL2 expression in 55%, of WR DLBCLs. BCL2, BCL6, IRF4 and MYC breakpoints were observed in, respectively, 3 of 42 (7%), 9 of 36 (25%), 2 of 26 (8%) and 4 of 40 (10%) contributive cases. A variable follicular pattern was evidenced in 30 of 68 (44%) large biopsy specimens. The 5-year progression-free survival (PFS) and the overall survival (OS) of 153 WR DLBCL patients with survival information were 69.5% and 77.8%, respectively. The GCB immunophenotype correlated with a better OS (P=0.0015), while BCL2 expression predicted a worse OS (P=0.037), an effect overcome by the GCB/non-GCB classification. Compared with matched nodal DLBCLs, WR DLBCLs with no age-adjusted international prognostic index factor disclosed a better 5-year PFS rate (77.5% versus 70.7%; P=0.03). Conclusions WR DLBCLs display distinct clinicopathologic features compared with conventional DLBCLs, with usual localized-stage disease, common follicular features and a high frequency of GCB immunophenotype contrasting with a low rate of BCL2 rearrangements. In addition, they seem to be associated with a better outcome than their nodal counterpart

Details

Database :
OAIster
Notes :
English
Publication Type :
Electronic Resource
Accession number :
edsoai.ocn999836096
Document Type :
Electronic Resource