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

Ann Oncol. 2012 Dec;23(12):3143-3151. doi: 10.1093/annonc/mds150. Epub 2012 Jun 13.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anthracyclines / therapeutic use
  • B-Lymphocytes / metabolism
  • B-Lymphocytes / pathology
  • DNA-Binding Proteins / metabolism
  • Disease-Free Survival
  • Female
  • Humans
  • Interferon Regulatory Factors / metabolism
  • Lymphoma, Large B-Cell, Diffuse / drug therapy
  • Lymphoma, Large B-Cell, Diffuse / mortality
  • Lymphoma, Large B-Cell, Diffuse / pathology*
  • Male
  • Middle Aged
  • Pharyngeal Neoplasms / drug therapy
  • Pharyngeal Neoplasms / mortality
  • Pharyngeal Neoplasms / pathology*
  • Proto-Oncogene Proteins c-bcl-2 / metabolism
  • Proto-Oncogene Proteins c-bcl-6
  • Proto-Oncogene Proteins c-myc / metabolism


  • Anthracyclines
  • BCL6 protein, human
  • DNA-Binding Proteins
  • Interferon Regulatory Factors
  • Proto-Oncogene Proteins c-bcl-2
  • Proto-Oncogene Proteins c-bcl-6
  • Proto-Oncogene Proteins c-myc
  • interferon regulatory factor-4