Miscellaneous Rare Malignancies: Intra-abdominal Lymphomas

Gastrointestinal lymphomas are a heterogeneous group of malignant diseases originating from the lymphoid tissue of the gastrointestinal system. Most lymphomas of the abdomen are the nodal form of disease, involving lymph nodes only, but only about one-fourth of gastrointestinal lymphomas are extranodal, that is, involving the gastrointestinal tube. Gastric lymphomas are the most common lymphomas of the gastrointestinal system. They account for about 3–5% of all gastric tumors. Some forms of gastric lymphomas are related to chronic H. pylori infection. Gastrointestinal lymphomas, either arising in the stomach, small bowel, or colon, present usually with nonspecific symptoms of abdominal pain, weight loss, and nausea/vomiting. Rarely they can present with an acute abdomen due to perforation or bleeding, or with obstructive symptoms. In an emergency, CT scan is the diagnostic modality of choice, but if the patient is stable enough, an endoscopy may help with the diagnosis. Emergency management is usually conservative, so that the patient can start chemotherapy as soon as possible, but surgery can have a role in complicated gastrointestinal lymphomas, in particular of the small bowel. In fact, in emergency, often it is difficult to have a precise diagnosis before the emergency laparotomy and patients presenting with a perforated mass of the bowel undergo laparotomy and resection well before a diagnosis of lymphoma is confirmed. In these cases, surgery has a double value of diagnosis and treatment. Although surgery has a role in the management of complications, long survival can be obtained only with chemotherapy and in the case of marginal zone lymphomas (MALT) with the eradication of H. pylori.