Primary Gastrointestinal T/NK Cell Lymphoma

Simple Summary Primary gastrointestinal T/NK cell lymphoma (GI-TNKL) is a heterogeneous group including various subtypes, anatomic locations, and clinicopathologic features. GI-TNKL is difficult to diagnose due to its rarity, various subtypes, and histopathological features. We performed a retrospective analysis of 38 cases of GI-TNKL in South Korea. GI-TNKL consisted of unique subtypes showing specific characteristics of macroscopy, histology, immunophenotype, and prevalent anatomic subsites. ENKTL and MEITL were relatively common. GI-TNKL showed aggressive behavior with short PFS and OS. This clinical and pathological descriptive analysis will be helpful for accurate understanding, diagnosis, and treatment. Primary gastrointestinal T/NK cell lymphoma (GI-TNKL) is an uncommon and heterogeneous group of lymphoid malignancies. We aimed to investigate their subtype distribution, clinicopathologic characteristics, and clinical outcomes. A total of 38 GI-TNKL cases and their clinical and pathological characteristics were analyzed. GI-TNKL occurred in adults with a median patient age in the sixth decade of life and showed a slight male predominance. The most common histologic type was extranodal NK/T-cell lymphoma, nasal type (ENKTL; 34.2%), followed by monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL; 31.6%), intestinal T-cell lymphoma, NOS (ITCL, NOS, 18.4%), anaplastic large cell lymphoma, ALK-negative (ALCL, ALK-; 13.2%). The small intestine was the primary affected region. More than 90% of patients complained of various GI symptoms and cases with advanced Lugano stage, high IPI score, or bowel perforation that required emergent operation were not uncommon. GI-TNKL also showed aggressive behavior with short progression-free survival and overall survival. This thorough clinical and pathological descriptive analysis will be helpful for accurate understanding, diagnosis, and treatment.

Automated summary

Primary gastrointestinal T/NK cell lymphoma (GI-TNKL) is a rare and heterogeneous group of lymphoid malignancies, with unique subtypes showing specific characteristics. Predominantly affecting adults, particularly males, the most common histologic types are ENKTL and MEITL, primarily in the small intestine. Patients typically present with GI symptoms, and cases with advanced disease may require emergency surgery. GI-TNKL exhibits aggressive behavior and has short progression-free survival and overall survival.