Intestinal diffuse large B-cell lymphoma: an evaluation of different staging systems

Abstract

The gastrointestinal tract is the most common primary extranodal site for diffuse large B-cell lymphoma (DLBCL). However, there is no consensus on the most appropriate staging system for intestinal DLBCL. We evaluated the utility of the modified Ann Arbor system, the Lugano system, and the Paris staging system (a modification of the Tumor, Node, Metastases [TNM] staging for epithelial tumors) in 66 cases of resected intestinal DLBCL. The cases were treated with surgery, plus either cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) chemotherapy alone (n=26) or with the addition of rituximab immunotherapy (n=40). Median follow-up time was 40.4 months (range, 2.1-171.6 months). Fifty-six patients (84.8%) achieved complete remission. The overall 5-yr survival rate was 86.4% (57/66). Of the stage categories defined for each staging system, only the T stage of the Paris classification showed prognostic significance for overall survival by univariate analysis. However, none of the stage parameters was significantly correlated with patient survival on multivariate analysis. In conclusion, the results suggest that the T stage of the Paris classification system may be a prognostic indicator in intestinal DLBCL. The results also imply that in surgically resected intestinal DLBCL, the addition of rituximab to the CHOP regimen does not confer significant survival advantage.

Keywords: Intestines; Lymphoma, Large B-Cell, Diffuse; Rituximab; Stage.

Fig. 1

Pathologic evaluation of depth of invasion and stage. Representative intestinal DLBCL cases involving mucosa and superficial submucosa (A, H&E, ×40), muscularis propria (B, H&E, scan view), subserosa (C, H&E, scan view), serosa (black arrows) (D, H&E, scan view), regional lymph node metastasis (E, H&E, ×40), and appendix non-contiguously (empty arrows) (F-H). (F) low magnification (H&E, ×40); (G) high magnification of region of interest in F (H&E, ×200); (H) CD20 immunostaining to identify the lesion (×200).

Fig. 2

Kaplan-Meier survival curves of overall survival according to clinical variables: (A) IPI risk group and (B) ECOG performance score.

Fig. 3

Kaplan-Meier survival curves of overall survival according to (A) T stage, (B) N stage, (C) M stage of the Paris classification system, and (D) modified Paris T stage. Note that the survival curves of the mucosa (M)/submucosa (SM) invasion group and the muscularis propria (PM) invasion group overlap completely. Abbreviations: M, mucosal confinement; PM, muscularis propria invasion; SM, submucosal invasion; SS, subserosal invasion; SI, serosa/adjacent organ invasion and/or perforation.