Gastrointestinal follicular lymphoma: using primary site as a predictor of survival

Abstract

Gastrointestinal follicular lymphoma (GI‐FL) is a rare extranodal variant of follicular lymphoma (FL) that has been increasingly reported in the literature. An especially indolent course is linked to the disease after a lack of observed patient death in past studies. However, overall survival (OS) and associated prognostic factors remain unclear. A large population‐based database was utilized to identify demographic and clinicopathologic characteristics of GI‐FL, along with survival differences among primary sites. The Surveillance, Epidemiology, and End Results Registry was used to identify GI‐FL cases between the years of 1973 and 2012. Kaplan–Meier curves compared OS differences and Cox proportional hazard models analyzed prognostic factors. Final analysis included 1109 cases. Small intestinal cases, which included those with single‐site and multi‐segment involvement, were most common (63.6%) followed by gastric (18.2%) and colorectal cases (18.2%). Small intestinal GI‐FL presented more frequently with grade I histology, and less often with grade III histology (P < 0.001 and P < 0.001, respectively). Small intestinal cases had better outcomes (5‐year OS = 80.9%, P < 0.001) compared to cases involving the stomach (5‐year OS = 52.7%) and colorectum (5‐year OS = 71.5%). On multivariate analysis for predictors of mortality, small intestinal involvement predicted for better survival; hazard ratio (HR) 0.66 (95% CI: 0.51–0.85). Advanced age (≥66), grade (grade III), and stage (Ann Arbor Stage III/IV) predicted for mortality with HR 5.46 (95% CI: 3.80–7.84), 1.42 (95% CI: 1.10–1.83), 1.57 (95% CI: 1.15–2.16), respectively. GI‐FL has poorer outcomes than previously suggested. Small intestinal involvement has a better prognosis. A possible biological basis for this will require further investigations in the future.

Primary duodenal follicular lymphoma with late disseminated nodal relapse responsive to rituximab monotherapy: A case report

“…Hence, follow-up evaluation after remission should include whole-body evaluation with PET scan or gallium scintigram. A recent analysis of the Surveillance, Epidemiology, and End Results database in the United States revealed that small intestinal involvement is an independent predictor of longer survival in GI-FL (13).…”

A case Report of Perforated Primary Follicular Lymphoma of the Jejunum presenting as Aneurismal Form

“…Studies show that small intestine is involved in 64 to 94% of cases [5]. Contrary to Lebrun and al [7] who found a special predilection of GI-FL in the terminal ileum, most of the latest GI-FL reported affected the duodenum [4,6,8]. But in many studies, small intestine follicular lymphomas have a predominantly multifocal nature.…”

“…Usually it's 9+B-cell lymphoma, and the diffuse large B-cell lymphoma (DLBCL) is the most frequent. Follicular lymphoma is the second most common subtype of B-cell lymphoma [2,4]. Primary GI-FL without peripherical lymphadenopathy is very rare.…”

“…Primary GI-FL without peripherical lymphadenopathy is very rare. Its incidence is estimated at 1-6% of all gastrointestinal NHL [4][5][6]. However, more cases have been reported in the literature in the last decades.…”

“…However, more cases have been reported in the literature in the last decades. It's why some authors argue that the incidence of GI-FL is higher [3,4]. The small intestine is the most involved site of GI-FL.…”