Primary gastrointestinal lymphomas.

Abstract

Recent evidence suggests that a significant proportion of primary gastrointestinal lymphomas are driven by exogenous agents/antigens. In the stomach, Helicobacter pylori appears to be responsible for most cases of low-grade lymphomas (MALToma), whereas an infectious etiology is suspected in immunoproliferative small intestine disease (IPSID). Similarly, enteropathy-associated T-cell lymphomas appear to result from a disordered response to gluten, although this profile remains controversial. Accordingly, although traditional antineoplastic treatments, such as surgery and radiation, are still important for the treatment of primary GI lymphomas, antibiotics may be the first line of therapy for low-grade gastric MALToma, and they are often used alone or in combination with chemotherapy for IPSID. In patients with celiac sprue, a gluten-free diet appears to markedly reduce the risk for lymphoma. An important caveat for the treatment of gastric lymphomas is that only low-grade gastric MALTomas have consistently responded to antibiotics. Treatment of high-grade gastric lymphoma is evolving. Although surgery was once considered central to diagnosis, staging, and treatment of gastric lymphoma, most patients can now have a diagnosis established by endoscopic biopsy and are candidates for chemotherapy and adjuvant radiation. The risks of fatal hemorrhage and perforation have probably been vastly overestimated and appear to be equal or less than the mortality associated with surgery. In addition, the long-term effects of gastric resection on quality of life have been almost completely ignored. Systemic lymphomas involve the GI tract far more often than is clinically apparent. In most cases, treatment should not be affected.