Small Intestine Cancer

The small intestine is approximately 20 feet long and consists of three sections: the duodenum, jejunum, and ileum. The small intestine performs extensive digestion and absorption functions. It also secrets secretin, which stimulates the pancreas to produce digestive enzymes.

Descriptive Epidemiology

Cancer of the small intestine is very rare; the age-adjusted incidence is approximately 1.4 per 100 000—less than 2% of all gastrointestinal malignancies. The incidence of small intestine cancer is higher in Maori of New Zealand and

Hawaiians, and it is lower in India, Romania, and other areas of Eastern Europe. In the United States, the incidences of adenocarcinoma, lymphoma, and carcinoid have only slightly increased since 1980s; even for lymphoma, which has had the largest increase, the annual rate of increase has been no more than 1 per 1 million.

Disease Process

There are four types of small intestine cancer, each with unique characteristics: adenocarcinoma, carcinoid, lymphoma, and sarcoma. In Western developed countries, approximately 30-40% of small intestine cancer is adenocarcinoma, predominantly in the duodenum, and carcinoid and lymphoma occur more often in the jejunum or ileum, whereas sarcoma may develop anywhere in the small intestine. In developed countries, lymphoma is very rare and occurs more often in older people with relatively good survival. In contrast, in developing countries, lymphoma is the main type of small intestine cancer, and it occurs more often in younger individuals, anywhere in the small intestine, with poor survival. Hence, prognosis of small intestine cancer depends on the type, geographic location (which may be an indication of etiology and/or the advancement of treatment), and disease stages. Clinical presentation may include abdominal pain, weight loss, abdominal mass, anemia, nausea/vomiting, bleeding, obstruction, jaundice, and anorexia before diagnosis. Overall, the 5-year survival rate is approximately 80% for carcinoid, 60% for lymphoma, 45% for sarcoma, and 20% for adenocarcinoma.

Risk Factors

Due to the rarity of small intestine cancer, etiologic investigation has relied on only a few small case-control studies. A lack of histology data has further undermined the strength of the evidence.

Tobacco use, alcohol consumption, and dietary factors such as high animal protein, high animal fat, sugar, and salted, cured, or smoked food were associated with small intestine cancer in some but not all studies. Small intestine adenoma, familial adenomatous polyposis, hereditary nonpolyposis colorectal cancer, peptic ulcer, celiac sprue, and cholescystectomy have been found to be associated with increased risk for small intestine adenocarci-noma. In people with Crohn's disease, a 16- to more than 100-fold increased risk for small intestine adenocarcinoma has been reported, but unlike most adenocarcinomas that occur in the duodenum, these patients tend to have adenocarcinomas in the elium. The reasons for the increased risk are uncertain, but it has been hypothesized to be due to the medication for treating Crohn's disease.


Because very little is known about the etiology of small intestine cancer, no preventive strategy has been proposed.

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