Sinonasal B‐cell lymphomas: A nationwide cohort study, with an emphasis on the prognosis and the recurrence pattern of primary diffuse large B‐cell lymphoma

Abstract

Lymphomas of the nasal cavity and paranasal sinuses (NPS) are rare. Knowledge on sinonasal B-cell lymphoma (SNBCL) primarily comes from case series or singlecenter studies on small cohorts. We sought to determine the subtype distribution, clinical characteristics, disease behavior, and prognosis on a nationwide scale, with an emphasis on prognostic factors for the most common sinonasal lymphoma, primary sinonasal diffuse large B-cell lymphoma (PSDLBCL). We collated all data from medical records and national databases on patients registered with SNBCL from 1980 through 2018 in the national pathology registry and collected all tissue samples for validation of diagnosis. We included 205 patients and found 10 different subtypes of lymphoma. Diffuse large B-cell lymphoma (DLBCL) was the predominant subtype (80%). The incidence of SNBCL was 0.14/100,000 personyears. The five-year progression-free survival (PFS) and overall survival rates for PSDLBCL were 50% and 56%, respectively. For PSDLBCL, Rituximab showed a statistically significant effect (Hazard Ratio 0.22, p < 0.001), whereas consolidative radiotherapy combined with immunochemotherapy was of limited value (PFS, p = 0.93). When treatment failure occurred, DLBCL showed a distinct pattern of recurrence/dissemination to the NPS, skin, breast, central nervous system (CNS), and/or testis. Collectively, DLBCL comprised a clear majority of SNBCLs, although nine other subtypes were represented. Data showed that immunochemotherapy increased survival for PSDLBCL and that the addition of radiotherapy did not benefit patients. Furthermore, treatment failure for sinonasal DLBCL showed a possible common pathogenesis with primary extranodal lymphomas of specific locations (e.g., CNS, skin, breast, and testis).This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

Characteristics of Extranodal NK/T-Cell Lymphoma, Nasal Type, Compared with Nasal Diffuse Large B-cell Lymphoma

“…In addition, patients with sinonasal DLBCL in Europe also had other different characteristics in other aspects from our results. In Europe, 3 the median age was higher than in our results (72.8 years), and nasal symptoms were more frequent than eye symptoms. Thus, additional research is needed to further elucidate the epidemiologic differences between ENKL and sinonasal DLBCL.…”

“…1,2 Diffuse large B-cell lymphoma (DLBCL) is the most common type and accounts for approximately 80% of all sinonasal lymphomas. 3 However, fast-growing malignant lymphomas, such as extranodal NK/T-cell lymphoma, nasal type (ENKL), must be considered as a differential diagnosis. 4,5 Extranodal NK/T-cell lymphoma, nasal type, an aggressive type of non-Hodgkin lymphoma (NHL), is derived from NK cells or γδ T-cells.…”

A rare case of sphenoid sinus lymphoma

“…The most common chemotherapy regimens include CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) and occasionally BACOP (bleomycin, doxorubicin, cyclophosphamide, vincristine, prednisolone) or CEOP (cyclophosphamide, epidoxorubicin, vincristine, prednisolone) 2 . Rituximab is frequently supplemented to the CHOP regimen with significant improvement in survival especially among women 2,18 . Surgery may be possible in select cases such as a focal tumor with clear margins or if the patient is symptomatic secondary to tumor burden 1,2,4 .…”

“…2 Rituximab is frequently supplemented to the CHOP regimen with significant improvement in survival especially among women. 2,18 Surgery may be possible in select cases such as a focal tumor with clear margins or if the patient is symptomatic secondary to tumor burden. 1,2,4 The majority of cases are not as responsive to surgery; however, when compared to a combination of chemotherapy and radiation.…”

Sinonasal lymphoma: A primer for otolaryngologists

“…The World Health Organization describes over 60 distinct NHL subtypes, of which 85%-90% arise from mature B lymphocytes and the remainder arise from mature T or natural killer (NKT) lymphocytes. 9,[24][25][26][27] Within the paranasal sinuses and nasal cavity, B-cell lymphomas carry a more favorable diagnosis, whereas NK/T cell lymphomas are traditionally associated with a rapid, deleterious F I G U R E 1 Preferred reporting items for systematic reviews and meta-analysis (PRISMA) flow diagram of study selection clinical course. 4 The two most common SN-NHL include diffuse large B-cell lymphoma (DLBCL), followed by extranodal natural killer/T-cell lymphoma, nasal type (ENKTL).…”