Clinicopathologic features and prognostic analysis of Waldeyer ring B-cell lymphoma

Abstract

This retrospective study is to explore the clinicopathologic, immunophenotypic, and molecular genetic features of Waldeyer ring B-cell lymphoma (WR-BCL). Tissue arrays from 65 WR-BCL cases were subjected to pathologic and immunophenotypic detections. Expression of Epstein–Barr virus-encoded small RNA (EBER) was detected by in situ hybridization. Interferon regulatory factor 4 (IRF4), BCL-2, BCL-6, and C-myelocytomatosis viral oncogeneav (MYC) gene abnormalities were investigated using interphase fluorescence in situ hybridization. Among the 65 patients, there were 12 nasopharynx cases, 49 tonsil cases, and 4 tongue root cases. Moreover, there were 49 cases of diffuse large BCL (DLBCL) and 16 cases of follicular lymphoma (FL). More than 60% of the patients had Ann Arbor stage III/IV disease, with infiltrated neighboring organs, invaded spleens, and increased lactate dehydrogenase (LDH) levels. Tumor cells were positive for multiple myeloma antigen 1 (MUM1), BCL-2, BCL-6, and C-MYC. EBER expression was detected in lymphoma cells of 2 cases. Alteration frequencies of IRF4, BCL-2, BCL-6, and C-MYC were 24.6%, 32.3%, 27.7%, and 30.7%, respectively. Approximately 67.69% cases had stages 0 to II disease, while 32.31% cases had stage III disease. Five-year overall survival rate was 65.12%. Eastern Cooperative Oncology Group performance status (ECOG) score ≥2 was the only adverse factor for overall survival. IRF4/MUM1, C-MYC, and CD10 expressions were related to poor disease prognosis. WR-BCLs were largely dependent on ECOG, LDH, and bone marrow involvement. WR-DLBCL was associated with poor survival outcomes compared with WR-FL. The WR-DLBCLs have distinct clinicopathologic features, with correlations between the IRF4/MUM1, C-MYC and CD10 expressions, ECOG, LDH, bone marrow involvement, and the disease prognosis.

Prevalence of IRF4 rearrangement in large B-cell lymphomas of the Waldeyer’s ring in adults

“…BCL2 rearrangements were demonstrated in half of the cases, sometimes with coexisting BCL2 -mutations. The rate of BCL2 translocations in our cohort was more than twice as high as in other studies investigating WR DLBCLs, depending on the comparison reference source, but matched very well with results from studies that included both nodal and extranodal LBCLs [ 15 , 18 , 20 ]. In the majority of the BCL2 -rearranged cases, an additional IGH translocation was demonstrated, suggesting the characteristic BCL2 :IGH translocation.…”

“…In this study, DLBCL of GCB-type was slightly below the rate reported in the WR in a previous study (46.7 vs 62%) [ 15 ]. However other groups have found even lower frequency of GCB-type DLBCL (20.4%) [ 18 ]. In general, unselected studies of extranodal and nodal DLBCL have reported GCB rates between 40 and 47% [ 2 , 19 ].…”

“…However, studies including only children and young adults have reported higher frequencies, between 21 to 32% of IRF4 rearranged cases, mainly involving the WR [ 23 , 24 ]. Only one study in adults has reported a relative high frequency (24.5%) of LBCL- IRF4 in the WR in a Chinese population [ 18 ]. The mean age of the Chinese cohort was lower when compared to our cohort (58 years vs 69 years); however, this does not explain the difference.…”

Clinico-Pathological Examination of Waldeyer Ring Lymphomas

“…As in our study, the rate of WR lymphomas among all lymphomas is around 10% (d 'Amore et al, 1991). Considering the gender distribution of WR lymphomas, the female and male ratios are close, but it is more common in females (Ma et al, 2020;Teh et al, 2014). However, in our study, the incidence rate in men was considerably higher than in women's.…”

“…The most common location of lymphomas in WR is tonsil, and tonsil location rates are over 50% (Ma et al, 2020;Teh et al, 2014). Although lymphomas most commonly originate from the tonsil in our study, the nasopharynx was the most common site.…”