Primary gastrointestinal (GI) non-Hodgkin lymphoma (NHL) is a heterogeneous group of B and T cell lymphoid malignancies. The clinical features, management, and prognosis of these lymphomas differ from lymphomas of lymph node origin.
Because GI NHLs are rare, optimal treatments have not been defined. Current recommendations are primarily based upon data from case series, rather than large, randomized clinical trials. The various treatment strategies for each of the predominant types of GI NHL will be reviewed here. The clinical manifestations and diagnosis of these tumors are discussed separately. (See "Clinical presentation and diagnosis of primary gastrointestinal lymphomas".)
After a diagnosis of GI lymphoma is confirmed, a pretreatment evaluation determines the extent of disease. The specific tests included depend partly upon the location of the tumor. In addition to a history and physical examination, it is our practice to perform the following pretreatment studies:
- Laboratory studies include a complete blood count with differential, HIV serology, chemistries with liver and renal function, electrolytes, and lactate dehydrogenase (LDH). Patients should undergo serologic testing for hepatitis B and hepatitis C. (See "Hepatitis B virus reactivation associated with immunosuppression".)
- A contrast-enhanced computed tomography (CT) scan of the chest, abdomen and pelvis should be performed to evaluate for distant disease. The use of positron emission tomography (PET) is controversial except in cases of diffuse large B cell lymphoma (DLBCL) [1-3]. (See "Evaluation and staging of non-Hodgkin lymphoma", section on 'Routine imaging studies'.)
- An endoscopic evaluation is performed based upon the site of disease. Patients with gastric lymphoma should undergo an esophagogastroduodenoscopy. Some institutions also perform endoscopic ultrasound to further determine the depth of invasion and involvement of perigastric lymph nodes . Patients with intestinal lymphoma should undergo endoscopy to evaluate the site of disease.
- Patients with gastric lymphoma should be tested for Helicobacter pylori (H pylori), which can be detected by histologic specimen, biopsy urease test, urea breath test, stool antigen test, or serology in the vast majority of gastric MALT lymphoma (90 percent) . In addition, fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR) testing for t(11;18) should be performed. (See "Indications and diagnostic tests for Helicobacter pylori infection".)
- Evaluation of nutritional and performance status. (See "The role of parenteral and enteral/oral nutritional support in patients with cancer".)
- Unilateral bone marrow biopsy and aspirate should be performed on all patients.
- Additional studies (eg, magnetic resonance imaging (MRI) of the orbit) should be reserved for select patients who have findings on history and/or physical examination concerning for involvement of additional sites.
- Men and women with childbearing potential should receive counseling about the potential effect of treatment on their fertility and options for fertility-preserving measures. (See "Fertility preservation in patients undergoing gonadotoxic treatment or gonadal resection".)
The Ann Arbor staging system used for most lymphomas is considered to be inadequate for the staging of GI lymphoma since it does not incorporate information on the depth of tumor invasion known to affect prognosis. Several other staging systems have been proposed, but with limited consensus. The lack of a uniform staging system has hindered the direct comparison of clinical trials. The most widely accepted staging system is the Lugano staging system.