Site-Specific Survival of Extra Nodal Diffuse Large B-Cell Lymphoma and Comparison With Gastrointestinal Diffuse Large B-Cell Lymphoma

Abstract

Background Diffuse large B-cell lymphoma (DLBCL) constitutes 30% of all non-Hodgkin’s lymphomas. It can present as a nodal disease or as an extra nodal disease. Based on the site of origin, extra nodal DLBCL (EN-DLBCL) may have a distinct clinical outcome. Apart from the site of origin, factors including demographics, stage, and presence of any other primary malignancy also affect the outcome. The purpose of our study was to characterize prognostically distinct groups based on the site of presentation of EN-DLBCL. Methods We used 18 registries in Surveillance, Epidemiology, and End Results database to identify the patients with EN-DLBCL for 2000 - 2015 with last follow-up till December 31, 2018. A total of 30,290 EN-DLBCL patients were selected and categorized based on 13 broad sites grouping. Demographic variables were summarized. We did overall survival analysis with univariate and multivariate Cox-proportional hazard modeling. Short-term survival trend was calculated as well. Results The percentage of EN-DLBCL of all DLBCLs is 34.48%. EN-DLBCL was comparatively seen more in males (54.94%) and non-Hispanic whites (71.52%). In terms of clinical characteristics, patients with EN-DLBCL were mostly diagnosed at age ≥ 60 years (66.11%), early stage (69.33%), and presentation as first primary cancer (81.89%). A higher risk of mortality was seen in non-Hispanic black (hazard ratio (HR) 1.36), with late age of onset (HR 2.69), late stage at presentation (HR 1.42), and with history of other malignancy (HR 1.29). Compared to the intestinal tract, the risk of overall mortality was higher in individuals with involvement of nervous system (HR 1.85), pancreas and hepatobiliary system (HR 1.22), and respiratory system (HR 1.18) and the best outcomes were seen in heart and mediastinal site (HR 0.58) of DLBCL. Conclusion Based upon our population-based study, we conclude that primary site of presentation of EN-DLBCL is an important prognostic factor with significant difference in survival based on histological and epidemiological characteristics.

Pancreatic Diffuse Large B-cell Lymphoma in the US Population

“…Higher mortality was observed in White patients and in patients with late presentation and a history of second malignancies. In comparison to intestinal tract DLBCL, a disease involving CNS, pancreas, and the respiratory system was associated with worse survival [ 12 ].…”

A Rare Case of Diffuse Large B-Cell Lymphoma Found in the Subcutaneous Scalp

“…The presentation described today is not often seen [2][3] and is considered a high-grade lymphoma [4][5]. Of all forms of DLBCL, extranodal forms only comprise 34.48% [10]. The gastrointestinal tract has been noted to be the most common extranodal site [8].…”

“…Rare sites of extranodal DLBCL have been documented in the literature, including presentations in the scalp, testicles, bone, bladder, and nasal septum [2][3][6][7][8][9]. A recent 2022 study found that the percentages of extranodal DLBCL from years 2000-2015 at the age of 18 or greater in their search were as follows: GI tract 28.31%, nervous system 13.78%, head and neck 11.79%, skin and soft tissue 9.54%, genitourinary 7.24%, musculoskeletal 5.80%, respiratory tract 5.17%, pancreas and hepatobiliary 4.16%, endocrine organs 3.66%, heart and mediastinum 3.28%, breast 2.49%, bone marrow 2.47%, and others 2.30% [10]. Extranodal DLBCL was found to be more common in non-Hispanic white males 60 years of age or older [10].…”

Diffuse Large B-Cell Lymphoma of the Colon in an Asymptomatic Patient

“…[4,5]. Among these extranodal sites, the GI tract is the most commonly involved site, especially the stomach in 65%, the small intestine in 20-35%, and the colon and rectum in 5-15% [6]. Typically, these patients present with gastrointestinal (GI) symptoms like abdominal pain, nausea, vomiting, weight loss, diarrhea, and rarely, can present as acute complete or partial bowel obstructions.…”