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The monomorphic variant appears to have a broader geographic distribution and is encountered in Asia and other regions where celiac disease is rare. The tumor forms an ulcerating mucosal mass that invades the wall of the intestine. The prognosis is usually poor with death frequently resulting from abdominal complications in patients already weakened by uncontrolled malabsorption.
Both classical EATL and the monomorphic form have a similar clinical course.