S2021 Clinical and Endoscopic Analysis of Primary T Cell Lymphoma of the Gastrointestinal Tract According to Pathology and EBV Status

Background:Primary gastrointestinal(GI) T-cell lymphomas are rare tumors and mostly reported from the East. The aims of this study were to determine the clinical and endoscopic features of primaryGI T cell lymphoma, with special emphasis on the pathologic classification, and to elucidate the correlation between Estein-Barr virus (EBV)/ T-cell receptor gene rearrangement (TCR) status and prognosis of this disease. Patients and Methods:35 cases of primary GI T cell lymphomas that satisfied Dawson's criteria were enrolled. The mean age at the time of diagnosis was 47.3 years (range 27 70), and the male:female ratio was 24:11. The 38 lesions from 35 patients were analyzed and classified into enteropathy-type (ETCL), NK/T cell (NK/T), peripheral T cell (PTCL), and anaplastic large cell (ALCL). Clinical characteristics, and endoscopic features were analyzed according to these types, and the survival analysis was done according to clinical factors and EBV/TCR status. EBV/TCR was determined by in situ hybridization and PCR. Results:The initial presenting symptoms were abdominal pain (n=18, 35.0%), fever (n=8, 21.0%), gastrointestinal bleeding (n=6, 15.8%), diarrhea (n=4, 10.5%), and soreness (n=2, 5.3%). As for the location, PTCL more commonly involved stomach (7/12, 58.3%) and duodenum (4/12, 33.3%) while ETCL occurred most frequently in small intestine (8/13, 61.5%), NK/T involved small (4/11, 36.4%) and large intestine (5/11, 45.6%) (p<0.05). The stage of disease at diagnosis was less advanced in NK/T than in ETCL and PTCL (p<0.05). Perforation developed more frequently in NK/T (46.2%) and ETCL (46.2%) than in PTCL (0%) (p<0.05). On endoscopy the lesions were ulcerative in 9 (33.4%), ulcerofungative in 7 (25.9%), ulceroinfiltrative in 5 (18.5%), infiltrative in 3 (11.1%), superficial/erosive in 2 (7.4%), and fungating in 1 (3.7%). The distribution was not different according to the pathologic types. The EBV positivity was higher in NK/T than other types (p<0.05). Initial treatment was surgical resection (n=22, 57.9%), chemotherapy (n=15,39.5%), or supportive care (n=1,2.6%). The median survival was 12.1 months. The poor prognosis is associated with more advanced stage, small bowel location, and perforation (p<0.05). Perforation was found to be the only independent predictor for the prognosis by multivariate analysis (p<0.05). Conclusion:Primary GI T cell lymphoma showed characteristic clinical features according to the 4 major pathologic types. The poor prognosis in this disease is associated with more advanced stage, small bowel location, and perforation. EBV is the most prevalent in NK/T cell type and is not affecting the prognosis.