Small intestine lymphoma - Gastrointestinal oncology

Small intestine lymphoma

Definition

A non-Hodgkin lymphoma which occurs within the small bowel particularly in the ileum.

Aetiology/pathophysiology

Non-Hodgkin B cell lymphoma arises in the mucosal associated lymphoid tissue (MALT) and occurs within the distal ileum.

T cell lymphomas form ulcerated plaques or strictures in the proximal small bowel. Coeliac disease predisposes to a T cell lymphoma, treatment with gluten free diets may reduce the risk.

A Burkitt lymphoma like tumour occurs in children from North Africa and the Middle East.

Immunoproliferative small intestine disease (IPSID) is a condition characterised by plasma cell proliferation in the lamina propria of the small bowel. IPSID occurs mainly in the Mediterranean and presents with malabsorption. Transformation into lymphoma may occur.

Perforation, bleeding and obstruction may occur with any form of lymphoma.

Management

Resection is performed where possible, radiotherapy and chemotherapy are often used postoperatively, although their efficacy is uncertain. Inoperable disease is treated by combination chemotherapy; radiotherapy is used for palliation. IPSID not complicated by lymphoma is treated with antibiotics.