Other Malignancy

Lymphoma

— single or multifocal, primary or secondary to nodal/systemic disease. Primary disease centres on the bowel with or without spread to regional nodes.

— MALToma: low/high-grade

B cell (70-90% of bowel lymphomas)

centrocyte-like cells with variable numbers of blasts (>20% = high-grade)

lymphoepithelial lesions monotypic immunoglobulin expression

±eosinophilia high-grade lesions are on a spectrum with diffuse large B-cell lymphoma.

— Burkitt's-type/B-lymphoblastic:

children (or adults with AIDS, non-endemic/non-EBV related) terminal ileum/ileocaecal valve—a high-grade lymphoma CD20, CD10, CD79a, tdt positive (lymphoblastic lymphoma). High (>90%) Ki67 index, starry sky appearance.

— multiple lymphomatous polyposis:

centrocytic lymphoma or mantle cell lymphoma—splenic, small and large bowel disease with numerous intestinal polyps an intermediate-grade lymphoma—aggressive disease with advanced stage at presentation CD20, CD5, cyclin D1 positive.

— EATCL: aggressive. 5% of GI lymphomas. Proximal jejunum. Pleomorphic medium to large cell infiltrate (CD3 positive) and adjacent enteropathic mucosa (atrophy/increased intraepithelial lymphocytes).

— follicle centre cell (follicular) lymphoma:

more usually spread from nodal disease rather than primary.

— immunoproliferative small intestinal disease (IPSID): Mediterranean countries alpha chain disease.

— post-transplant lymphoproliferative disorders: polyclonal/monclonal/disparate morphology and behaviour some regress on decreasing immunosuppression therapy, e.g. cyclo-sporin (see Chapter 35).

Prognosis is better for low-grade B cell lymphomas (44-75% 5-year survival) than high-grade B- or T-cell lymphomas (25-37% 5-year survival). Adverse prognostic indicators are perforation, high-grade histology, multiple tumours, large size, serosal penetration and advanced stage (Ann Arbor System, see Chapter 35).

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