A New and Validated Clinical Prognostic Model (EPI) for Enteropathy-Associated XXXX .

@entity1 @entity894 @entity784 @entity3191 @entity1109 @entity1893
PURPOSE: @entity784 (EATL) is a rare intestinal @entity1893 with a poor, though variable prognosis. The International Prognostic Index (IPI) and the prognostic index for @entity3191 ( @entity3191 ) have limited predictive value for outcome of EATL. The purpose of this study was to develop and validate a prognostic model for EATL, which can identify high-risk @entity1 who need more aggressive therapy. EXPERIMENTAL DESIGN: This retrospective multicenter study was based on 92 @entity1 and included 45 @entity1 diagnosed with EATL between 1999 and 2009 from the Netherlands and 47 @entity1 from England and Scotland, diagnosed with EATL between 1994 and 1998. A new @entity894 (EPI) was constructed using the RPART (recursive partitioning and regression trees) procedure. Validation was performed applying the bootstrap method. RESULTS: Three risk groups were distinguished (P < 0.0001): a high-risk group, characterized by the presence of @entity1109 [median overall survival (OS) of 2 months]; an intermediate-risk group, comprising @entity1 without @entity1109 and an IPI score >=  2 (7 months); and a low-risk group, representing @entity1 without @entity1109 and an IPI score of 0 to 1 (34 months). Internal validation showed stability of statistical significance and prognostic discrimination. In contrast with the IPI and @entity3191 , the EPI better classified @entity1 in risk groups according to their clinical outcome. CONCLUSIONS: Our new, validated, prognostic model EPI accurately predicts survival outcome in EATL and may be used for @entity1 selection for new therapeutic strategies and evaluation of clinical trials. Clin Cancer Res; 21(13); 3013-9.   2015 AACR.
