Clinical features and outcomes of diffuse large B-cell lymphoma based on nodal or extranodal primary sites of origin: Analysis of 1,085 WHO classified cases in a single institution in China

Abstract

ObjectiveTo explore the clinicobiologic features and outcomes of diffuse large B-cell lymphoma (DLBCL) patients in China according to the primary site.MethodsA total of 1,085 patients diagnosed with DLBCL in National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College during a 6-year period were enrolled. Their clinical characteristics and outcomes were analyzed according to the primary site.ResultsIn the 1,085 patients, 679 (62.6%) cases were nodal DLBCL (N-DLBCL) and 406 cases (37.4%) were extranodal DLBCL (EN-DLBCL). The most common sites of N-DLBCL were lymphonodus (64.8%), Waldeyer’s ring (19.7%), mediastinum (12.8%) and spleen (2.7%), while in EN-DLBCL, stomach (22.4%), intestine (16.0%), nose and sinuses (8.9%), testis (8.4%), skin (7.9%), thyroid (6.9%), central nervous system (CNS) (6.4%), breast (5.7%), bone (3.4%), and salivary gland (2.7%) were most common. N-DLBCL patients tend to present B symptoms, bulky disease, and elevated LDH more often, while age >60 years, extranodal sites >1, Ann Arbor stage I or II, bone marrow involvement, and Ki-67 index >90% were usually seen in EN-DLBCL. The 5-year overall survival (OS) rate and progression-free survival (PFS) rate for all patients were 62.5% and 54.2%. The 5-year OS rate for patients with N-DLBCL and EN-DLBCL were 65.5% and 56.9% (P=0.008), and the 5-year PFS were 57.0% and 49.0% (P=0.020). Waldeyer’s ring originated DLBCL possessed the highest 5-year OS rate (83.6%) and PFS rate (76.9%) in N-DLBCL. The top five EN-DLBCL subtypes with favorable prognosis were stomach, breast, nose and sinuses, lung, salivary gland, with 5-year OS rate: 70.3%, 69.6%, 69.4%, 66.7% and 63.6%, respectively. While CNS, testis, oral cavity and kidney originated EN-DLBCL faced miserable prognosis, with 5-year OS rate of 26.9%, 38.2%, and 42.9%.ConclusionsIn our study, primary sites were associated with clinical characteristics and outcomes. Compared with EN-DLBCL, N-DLBCL had better prognosis.

R‐CHOP immunochemotherapy plus surgery is associated with a superior prognosis in Chinese primary intestinal diffuse large B‐cell lymphoma

“…Our previous studies suggest that the prognosis of EN-DLBCL is worse compared with that of N-DLBCL. 10 The 5-year OS rates for patients with N-DLBCL and EN-DLBCL receiving R-CHOP chemotherapy were 65.5% and 56.9%, and the 5-year PFS were 57.0% and 49.0%,respectively. Primary gastric and intestinal DLBCL displayed a different biological behavior and various patterns of antigen expression, but the standard chemotherapy remains the same with that for other N-DLBCLs.…”

“…Therefore, patients with EN-DLBCL appear to have a poorer prognosis compared with those with N-DLBCL. 10 The stomach and intestine are the most common extranodal organs affected by lymphoma. The incidence of primary gastrointestinal (GI) lymphoma is about 30-50% among all NHL cases.…”

Genome-wide Screens Identify Lineage- and Tumor Specific-Genes Modulating MHC-I and MHC-II Immunosurveillance in Human Lymphomas

“…Tumors without such genetic immunoevasion strategies are likely highly immunogenic and must escape from T cell pressure in other ways -their frequent development in extranodal (and often immune privileged) sites is well established (Bruno et al, 2014;Kraan et al, 2013;Shi et al, 2019). Together, these findings might help explain the disappointing clinical outcomes in single-agent immune checkpoint blockade trials for DLBCL (Ansell et al, 2019;Lesokhin et al, 2016) and indicate that NK-mediated or CAR-T immunotherapies may be better suited for ABC DLBCL immunotherapies.…”

Nomogram for Predicting the Overall Survival of Adult Patients With Primary Gastrointestinal Diffuse Large B Cell Lymphoma: A SEER- Based Study

“…We also observed this demographic feature in the present study. More and more studies showed that primary GI DLBCL had different clinical characteristics and treatment outcomes from nodal DLBCL ( 13 – 15 ). Some new prognostic evaluation systems are needed to identify high-risk patients.…”