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

Objective: To explore the clinicobiologic features and outcomes of diffuse large B-cell lymphoma (DLBCL) patients in China according to the primary site.

Methods: A 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.

Results: In 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%.

Conclusions: In our study, primary sites were associated with clinical characteristics and outcomes. Compared with EN-DLBCL, N-DLBCL had better prognosis.

Keywords: Diffuse large B-cell lymphoma (DLBCL); distribution; extranodal; nodal.

1

Proportion of diffuse large B-cell lymphoma by age.

2

Distribution of primary sites in nodal diffuse large B-cell lymphoma (N-DLBCL) (A) and extranodal diffuse large B-cell lymphoma (EN-DLBCL) (B). CNS, central nervous system.

3

Overall survival (OS) (A) and progression-free survival (PFS) (B) for patients with nodal diffuse large B-cell lymphoma (N-DLBCL) and extranodal diffuse large B-cell lymphoma (EN-DLBCL). (A) P=0.008; (B) P=0.020.

4

Overall survival (OS) (A) and progression-free survival (PFS) (B) for patients with nodal diffuse large B-cell lymphoma (N-DLBCL) according to the primary site of the lymphoma. (A) P<0.001; (B) P<0.001.

5

Overall survival (OS) (A) and progression-free survival (PFS) (B) for patients with extranodal diffuse large B-cell lymphoma (EN-DLBCL) according to the primary site of the lymphoma. (A) P=0.025; (B) P=0.030. CNS, central nervous system.