Clinical features

Symptoms produced by small intestinal lymphomas depend upon the specific histological type. Indolent lymphomas of B-cell lineage typically present with abdominal pain, weight loss and bowel obstruction {424}. Occasional cases present with nausea and vomiting, while rare cases are discovered incidentally. More aggressive tumours, such as those of T-cell lineage (described separately) or Burkitt lymphoma, may present as a large intra-abdominal mass or acutely with intestinal perforation. IPSID often manifests as abdominal pain, chronic severe intermittent diarrhoea and weight loss {1649}. The diarrhoea is mainly the result of steatorrhoea, and a protein-losing enteropathy can be seen. Peripheral oedema, tetany and clubbing are observed in as many as 50% of patients. Rectal bleeding is uncommon in small bowel lymphoma, but a common presenting sign in primary colonic lymphoma.

Burkitt lymphoma is most frequently seen in the terminal ileum or ileocaecal region, and may cause intussusception.

Imaging and endoscopy

Radiological studies are useful adjuncts to the diagnosis of small intestinal lymphomas, including barium studies and computerized tomography scans. T-cell lymphomas are typically localized in the jejunum, presenting as thickened plaques, ulcers, or strictures. Most B-cell lymphomas manifest as exophytic or annular tumour masses in the ileum {792}. B-cell lymphomas of both low- and intermediate-grade may produce nodules or polyps that can be seen both endoscopi-cally and by imaging. Most small intestinal lymphomas are localized to one anatomic site, but multifocal tumours are detected in approximately 8% of cases. Multiple lymphomatous polyposis consists of numerous polypoid lesions throughout the gastrointestinal tract {791}. Most often, the jejunum and terminal ileum are involved, but lesions can appear in the stomach, duodenum, colon, and rectum. This entity produces a characteristic radiological picture that is virtually diagnostic. As discussed below, the majority of such cases is caused by mantle cell lymphoma, but other subtypes of lymphoma may produce a similar radiological pattern {1034}.

IPSID. The macroscopic appearance of IPSID depends on the stage of disease. Early on, the bowel may appear endo-scopically normal, with infiltration appar

ent only on Intestinal biopsy. The disease may then progress to thickening of the upper jejunum together with enlargement of the mesenteric lymph nodes and the development of lymphomatous masses. Typically, the spleen is not involved and may even be small and fibrotic, as described in coeliac disease. Distal spread beyond the abdomen is uncommon {1649, 798}.

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