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Intestinal T-cell lymphoma (with and without enteropathy)
A tumor of intraepithelial T-lymphocytes, showing varying degrees of transformation but usually presenting as a tumor composed of large lymphoid cells. Tumor most commonly occurs in the jejunum or ileum. This disease is most commonly found in the setting of celiac disease.
Often preceded by celiac disease (gluten sensitive enteropathy) which is sometimes accompanied by intestinal ulceration (ulcerative jejunitis), but this is not a true "precursor" or "transformation."
In 10-20% of cases the lymphoma is composed of monomorphic medium-sized cells. This monorophic variant may be called Type II EATL.
Enteropathy-associated T-cell lymphoma (EATL) occurs most commonly in the jejunum or ileum. Presentation in the duodenum, stomach, colon, our outside the GI tract may occur, but rarely. A small proportion of the patients have a history of childhood onset celiac disease. Most show adult onset disease or are diagnosed as having celiac disease in the same clinical episode in which the lymphoma is diagnosed. Patients present with abdominal pain, often associated with intestinal perforation. In a proportion of patients there is a prodromal period of refractory celiac disease that is sometimes accompanied by intestinal ulceration (ulcerative jejunitis). This disease is uncommon in most parts of the world, but is seen with greater frequency in those areas with a high prevalence of celiac disease, in particular Northern Europe. 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. Recurrences are most frequent in the small intestine. Both classical EATL and the monomorphic form have a similar clinical course.
Definitive Diagnostic Methods
8q24(MYC) amplifications (monomorphic variant)
Chromosome gains in 1q and 5q
Complex segmental amplifications of 9q31.3-qter chromosome region
HLADQA1*0501, DQB1*0201 genotype
T-cell receptor (TCR) Beta and Gamma (TRB@, TRG@) genes are clonally rearranged