Diffuse large B-cell lymphoma of Waldeyer's ring has distinct clinicopathologic features: a GELA study.
BACKGROUND
Diffuse large B-cell lymphomas (DLBCLs) arising in specific extranodal sites have peculiar clinicopathologic features.


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.
Department of Laboratories, Institute of Pathology, C.H.U.V. Lausanne, Lausanne, Switzerland. Electronic address: laurence.deleval@chuv.ch.
L
L
de Leval
Department of Clinical Hematology, C.H.U. of Liège, Liège, Belgium.
C
C
Bonnet
Lymphoid Malignancies Unit, Henri-Mondor Hospital, AP-HP, Créteil; INSERM U955, Henri-Mondor Hospital, Créteil; Department of Medicine, Paris-Est University, Créteil, France.
C
C
Copie-Bergman
Department of Biostatistics, Liège University, Liège, Belgium.
L
L
Seidel
Lymphoid Malignancies Unit, Henri-Mondor Hospital, AP-HP, Créteil; INSERM U955, Henri-Mondor Hospital, Créteil.
M
M
Baia
INSERM U728, Saint-Louis Hospital, Paris; Department of Pathology, Saint Louis Hospital, AP-HP, Paris.
J
J
Brière
Department of Pathology, Hôtel-Dieu Hospital, AP-HP, Paris Descartes University, Paris.
T J
TJ
Molina
Department of Pathology, Saint-Antoine Hospital, Paris.
B
B
Fabiani
Department of Pathology, C.H.U., Dijon.
T
T
Petrella
Department of Biopathology, Morpological Unit, Gustave Roussy Institute, Villejuif, France.
J
J
Bosq
INSERM U728, Saint-Louis Hospital, Paris.
C
C
Gisselbrecht
Institute of Human Genetics, Christian-Albrechts-University, Kiel; University Hospital Schleswig-Holstein, Campus Kiel, Kiel, Germany.
R
R
Siebert
Department of Hematology, UMR918, Henri Becquerel Center, Rouen University, Rouen, France.
H
H
Tilly
Lymphoid Malignancies Unit, Henri-Mondor Hospital, AP-HP, Créteil; INSERM U955, Henri-Mondor Hospital, Créteil; Department of Medicine, Paris-Est University, Créteil, France.
C
C
Haioun
Department of Clinical Hematology, C.H.U. of Liège, Liège, Belgium.
G
G
Fillet
Lymphoid Malignancies Unit, Henri-Mondor Hospital, AP-HP, Créteil; INSERM U955, Henri-Mondor Hospital, Créteil; Department of Medicine, Paris-Est University, Créteil, France.
P
P
Gaulard
D018943:Anthracyclines / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000378:metabolism / Q000473:pathology; D004268:DNA-Binding Proteins / Q000378:metabolism; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D050835:Interferon Regulatory Factors / Q000378:metabolism; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010610:Pharyngeal Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000378:metabolism; D051560:Proto-Oncogene Proteins c-bcl-6; D016271:Proto-Oncogene Proteins c-myc / Q000378:metabolism; D000099218:Interferon Regulatory Factor-4
D018943:Anthracyclines; D004268:DNA-Binding Proteins; D050835:Interferon Regulatory Factors; D019253:Proto-Oncogene Proteins c-bcl-2; D051560:Proto-Oncogene Proteins c-bcl-6; D016271:Proto-Oncogene Proteins c-myc; D000099218:Interferon Regulatory Factor-4; C084103:BCL6 protein, human