The small intestine has a remarkably low incidence of primary carcinomas, especially considering its size. Those that do occur are often related to genetic syndromes, especially familial adenomatous polyposis.

Lymphomas and endocrine tumours are as frequent as carcinomas and have important associations with precursor conditions such as coeliac sprue, multiple endocrine neoplasia and Von Recklinghausen Syndrome.

The small intestine is the main site for metastatic tumours in the gastrointestinal tract.

WHO histological classification of tumours of the small intestine1

Epithelial tumours

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