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Gastrointestinal Lymphoma

In systemic dissemination of non-Hodgkin lymphoma (see chapter 13), any segment of the gastrointestinal tract may be involved again. However, up to 40% of lymphomas develop not in the lymph nodes, but in other organs, among which the intestine is the most frequent localization. By the time of recognition of the primary lymphoma of the gastrointestinal tract, the tumor process does not affect the liver, nor the spleen, nor the bone marrow. But regional lymph nodes can be affected. Primary gastrointestinal lymphomas usually occur as sporadic (single) tumors. They are often found in chronic malabsorption syndromes, congenital immunodeficiency states. HIV infections, iatrogenic immunosuppression after organ transplantation. Special attention should be given to lymphomas in the indigenous inhabitants of the Mediterranean region.

Sporadic lymphomas. Of all the primary digestive tract lymphomas, they are the most common form in the Western Hemisphere. These B-cell lymphomas (see Chapter 13) come from B-cells of the lymphoid tissue associated with the mucous membrane (MALT) and differ from their counterparts growing from the lymph nodes in three ways. In the early stages, they manifest themselves as local nodes and lend themselves to surgical removal; if relapse occurs, then exclusively in the gastrointestinal tract; genotype changes in these tumors differ from the corresponding changes in lymphomas of the lymph nodes. Unlike systemic ones, there are no translocations of t (14; 18) and t (ll; 14) in intestinal lymphomas. At the same time, unusual distributions of the c-tus gene may prevail in them (see Chapter 8). This type of gastrointestinal lymphoma usually affects adults and can occur in any part of the digestive tube: in the stomach (55-60%), small intestine (25-30%), proximal (10-15%) and distal (up to 10%) colon. The appendix and esophagus are extremely rarely affected. Although sporadic lymphomas are not associated with other diseases, there is a concept that these lymphomas develop with chronic activation of the lymphoid tissue of the intestinal mucosa. And such activation can cause chronic gastritis caused by Helicobacter pylori. The prognosis for sporadic intestinal lymphoma is better than for other organ forms of this tumor. The 10-year survival rate of patients with a localized process reaches 85%.

Spru-associated lymphomas (associated with malabsorption). They develop in some patients with long-existing malabsorption syndrome, which in turn may or may not be true glutensensitive enteropathy. Such lymphomas affect relatively young people (30–40 years old), often suffering from malabsorption for 10–20 years. There is a "reverse" option, when diffuse enteropathy with malabsorption accompanies the development of intestinal lymphoma. In most patients, sprue-associated lymphoma appears in the proximal small intestine.
Since it is almost always a T-cell tumor, the prognosis is poor.

Mediterranean lymphoma. This lymphoma refers to an unusual intestinal B-cell tumor. Against the background of chronic diffuse plasmacytosis (an increase in the number of plasma cells) of the intestinal mucosa, this lymphoma occurs in children, young and adult people who have ancestors from the Mediterranean region. Plasma cells produce abnormal heavy a-chains, the synthesis of one or another part of which is delayed. In a large number of patients, malabsorption precedes the appearance of intestinal lymphoma. This disease, also called immunoproliferative disease of the small intestine, has a poor prognosis.

Consider the morphological features of the lymphomas of the gastrointestinal tract. The macroscopic appearance of such lymphomas varies. Since the lymphatic tissue is located in the mucous membrane or submucosa of the intestine, in the early stages the tumor has the form of a plaque-shaped node. Then diffuse-infiltrative growth leads to thickening of the wall with smoothing of the folds of the mucous membrane and focal ulcerations. Sometimes there are either polypoid growth in the lumen of the organ, or mushroom masses with ulceration. The germination of the muscle of the intestine occurs with the destruction of muscles. Large tumor nodes cause peristalsis and obstruction. Deprived of adequate stromal support, such nodes can necrotic, due to which perforation and peritonitis occur. The decrease in tumor mass due to chemotherapy is also accompanied by perforation.

Under the microscope, in the early stages of lymphoma growth, atypical lymphoid elements that infiltrate the mucous membrane can be found. The expansion of lymphoid tissue in this membrane is quite pronounced, along with this there is a flattening and loss of glands. A large number of atypical elements can penetrate the surface epithelium. As the progression in the affected area, the mucous membrane, submucosa, and sometimes muscle layers are replaced by an infiltrate of relatively monomorphic malignant cells. The population of these cells has a mixed composition: in various ratios, it determines the small elements of the follicular centers (split and non-split, see chapter 13) and immunoblasts. Occasionally, lymphoid follicles can be found. It is known that the majority (more than 95%) of intestinal lymphomas belong to B-cell tumors. They are divided into high and low differentiated. In individuals, T-cell intestinal lymphomas occur. They are only highly differentiated. The prognosis is better for stomach lymphomas, worse for similar neoplasms growing in any part of the intestine. As for other tumors, the most important prognostic parameters are the depth of invasion, the mass (volume) of the tumor node, the degree of histological differentiation and the level of tumor spread.

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Gastrointestinal Lymphoma

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