Gastrointestinal lymphoma in tertiary gastroenterology center: clinical, endoscopic and pathological features.

Abstract

INTRODUCTION: Within the heterogeneous group of extranodal lymphoma (EL), the gastrointestinal tract (GT) is the most frequently involved extranodal site accounting for 30-50% of all extranodal cases [1,2]. Gastrointestinal involvement most often occurs secondarily, while the primary gastrointestinal lymphomas (GL) are relatively rare accounting for 30%-45% of all EL [3] and 0.9% of all GT tumors [4]. Lymphoma can arise from any localization within the GT, but the stomach is the most commonly involved organ being affected in 50-70% of all the GL, followed by the small intestine, ileocecal region, colon and esophagus [5-7]. Diffuse large B-cell lymphoma (DLBCL) and the marginal zone B-cell lymphoma (MALT type) are the most frequent histological types among the primary EL [8]. The aim of the study was to analyze and present data regarding the epidemiological and clinicopathological features of the patients with GL diagnosed in our institution for the analyzed period. MATERIAL AND METHODS: We retrospectively reviewed the medical records of patients with primary or secondary GL diagnosed at our clinic over a fifteen year period (January 1, 1999 to December 31, 2013). We analyzed the demographic data, clinical presentation, anatomic distribution, endoscopic aspect of the lesion, extension of the neoplastic process, occurrence of different histological subtypes, modalities of treatment and outcomes. RESULTS: We discovered 18 patients with GL, 7 males (39%) and 11 females (61%). Fourteen patients (77.7%) presented with dominant gastrointestinal involvement and were considered primary GL, while 4 patients (22.2%) had been previously diagnosed with generalized nodal disease additionally relapsing within the GT and were considered secondary GL. The stomach was the most prevalent extranodal localization being affected in 14 cases (11 primary and 3 secondary GL), there were 2 duodenal lymphoma, 1 lymphoma of the terminal ileum and 1 peritoneal lymphoma. Six of the gastric lymphoma were infiltrating the corpus, in 5 cases there was infiltration of the corporal and proximal gastric segment, in 2 cases the gastric antrum and pyloric region were involved and one case was restricted to the subcardial region. In most patients (10) massive and diffuse gastrointestinal infiltration was diagnosed, 5 patients had ulcerated lesions in the stomach and 3 patients presented with polyploid mass. Six patients with gastric lymphoma presented with upper gastrointestinal bleeding, 1 patient with duodenal lymphoma presented with biliary tract obstruction, the patient with the small bowel lymphoma presented with protein losing enteropathy, malabsorption and latter developed bowel perforation and the patient with peritoneal lymphoma presented only with ascites and pleural effusion. All the malignant lymphoma were Non-Hodgkin type and among them we registered only one T cell lymphoma. There were 6 DLBCL, 4 MALT lymphomas, 3 lymphocytic lymphomas, 1 anaplastic large cell lymphoma, 1 precursor B lymphoblastic leukemia/lymphoma, 1 peripheral T-cell lymphoma, anaplastic type (the lymphoma of the terminal ileum) and 2 lymphomas were not classified at the time being. The lymphoma was limited to the GT in 6 patients, 7 patients had regional nodal involvement, in 2 patients there was an intra-abdominal spread of the neoplastic process and in 3 patients there was an extra-abdominal dissemination. Most patients received chemotherapy and only 2 patients were treated surgically. Two patients (aggressive peritoneal DLBCL and peripheral T-cell lymphoma of the terminal ileum) had rapidly progressive clinical course and lethal outcome shortly after the diagnosis was established and before chemotherapy was administered. CONCLUSION: The GL has a variable clinical presentation and endoscopic aspect that often makes the diagnosis challenging. Substantial level of diagnostic awareness and comprehensive clinical approach are necessary in order to establish the diagnosis correctly, provide appropriate treatment and prolong survival.