AbstractBackgroundThe gastrointestinal (GI) tract is the second most frequent extranasal involvement site for ENKTL. This study aimed to explore the clinicopathological features, treatment models, survival outcomes, and prognosis of gastrointestinal ENKTL (GI‐ENKTL).MethodsThe clinical data of GI‐ENKTL patients were extracted from the China Lymphoma Collaborative Group (CLCG) database and were analyzed retrospectively.ResultsA total of 30 patients were enrolled, with a male/female ratio of 4:1 and a median age of 42 years. Twenty‐nine patients received chemotherapy, of whom 15 patients received asparaginase‐based (ASP‐based) regimens. Moreover, seven received surgery and three received radiotherapy. The overall response an d complete remission rates were 50.0% and 30.0% for the whole cohort, 50.0% and 37.5% for patients treated with ASP‐based regimens, and 50.0% and 25.0% for those treated with non‐ASP‐based regimens, respectively. The median follow‐up was 12.9 months and the 1‐year overall survival rate was 40.0% for the whole cohort. For those patients in an early stage, ASP‐based regimens resulted in a superior 1‐year progression‐free survival rate compared to non‐ASP‐based regimens (100.0% vs. 36.0%,p= .07). However, ASP‐based regimens did not improve survival in patients at an advanced stage.ConclusionGI‐ENKTL still has a poor prognosis, even in the era of modern asparaginase‐based treatment strategies.