Statistics from Altmetric.com
Since mucosa associated lymphoid tissue (MALT) lymphomas were first recognised as a distinct entity in 1983, their characterisation and management have seen a series of rapid advances. Here we concentrate on MALT lymphomas at the commonest site—the stomach. Historically, despite their indolence, “localised” gastric low grade lymphomas were treated with surgery, and chemotherapy or radiotherapy were reserved for systemic spread. This emphasis has gradually changed but the major advance came with the discovery that these tumours were caused by Helicobacter pylori infection, and the remarkable finding that most resolved with its treatment.1H pylori eradication quickly became a firstline therapy, and the research focus changed to how best to assess which cases would respond to this. Endoscopic ultrasound became important for staging and, to some extent, stage predicted response to H pylori eradication. However, the most exciting research over the past eight years has been in understanding the molecular genetics of these fascinating tumours. In this review, we describe these advances and discuss their implications for management, particularly the extent to which they predict the biological behaviour and response of the tumour to H pylori treatment.
CLASSIFICATION AND PATHOLOGY
The term MALT lymphoma was first suggested by Isaacson and Wright in 1983 to describe low grade gastric B cell lymphoma and immunoproliferative small intestinal disease.2 It was soon extended to include similar lymphomas at other mucosal sites such as the lung, occula adnexa, thyroid, and salivary glands. Later the World Health Organisation renamed these tumours “Extranodal marginal zone B cell lymphomas of mucosa associated lymphoid tissue” but also retained the shortened term “MALT lymphoma”.3 The stomach is by far the commonest site for MALT lymphomas, comprising 70% of the total. Despite this, gastric MALT lymphoma is an unusual disease, with an incidence of approximately 0.8 per 100 000 per year. …