Background
Deriving from lymphoid tissue beneath mucosa of gastrointestinal wall, primary gastrointestinal lymphoma (PGIL) was extranodal non-Hodgkin’s lymphomas (NHL) that account for 24–49 % of NHL [
1]. It used to appear in the stomach and small intestines, accounting for 1–4 % of gastroenteric tumor [
2]. PGIL was difficult to identify because clinical features of PGIL was indistinguishable from other gastroenteric diseases, especially from gastrointestinal tumor, which attributes to a high misdiagnosis rate in clinical practice. Besides, the treatment methods were quite different from each other. A retrospective analysis was made on 87 PGIL patients from January 1999 to December 2010 in the First Affiliated Hospital of Dalian Medical University; we summarized their clinical features, pathological patterns, treatment methods, and prognosis factors.
Discussion
Although PGIL pathogenesis remained unknown, certain factors had been considered to be related with its incidence for a long time, including virus infection, autoimmune deficiency, and environment pollution. [
3,
4]. The incidence of PGIL had increased in Asia, North America, and Europe [
5‐
8]. Many studies showed that the stomach was the most commonly involved site followed by the intestine, while in Pacific Ocean, small intestines were mostly seen followed by the stomach and colon [
9,
10]. In our study, we found that 53.9 % patients were PGL and 46.1 % were PIL. Furthermore, the majority of PGL were located at the antrum of the stomach, followed by the body of the stomach, and the most frequent site of PIL was the small intestine, especially within 100 cm far away from ileocecal valve. Besides, it should be noticed in clinical work that there might be multiple lesion locations in PGIL.
Clinical symptoms of PGIL were indistinguishable from other digestive system diseases. The main symptom included abdominal pain or discomfort, together with weight loss and nausea and other intestinal symptoms [
11,
12]. Imageological examination might show wall thickened and intestinal masses; it was usually difficult to identify from other gastrointestinal cancer. Endoscopy and biopsy were the most reliable methods for confirming diagnosis [
13,
14].
The surgical treatment was traditionally considered as the main treatment methods of PGIL. Most of patients accepted the radical resection. Palliative resection might due to huge size of tumor or extensive transfer of lymph node. However, as lymphoma was highly sensitive to chemotherapy, the main treatment of PGIL was non-surgery now. A prospective study showed that surgery treatment could not improve the 10-year survival rate of PGIL by comparing of surgery plus chemotherapy with chemotherapy alone [
6]. Recently, there was a study showed that it had equivalent efficacy whether patients accepted operation or not [
11]. Moreover, more and more studies demonstrated that non-surgery strategies had better OS [
15,
16]. In our study, 50 patients accepted non-surgery methods, such as CHOP or R-CHOP, which account for 54.5 % of total patients. Rituximab is a chimeric monoclonal antibody against the protein CD20, which is primarily found on the surface of immune system B cells. Rituximab destroys both normal and malignant B cells that have CD20 on their surfaces. The addition of rituximab has improved the overall survival of lymphoma. Many studies have showed that rituximab can improve the efficacy of chemotherapy after relapse [
17,
18]. In our retrospective study, there were not all patients whose CD20-positive approved rituximab therapy due to economic reasons. Nowadays, surgery had gradually been replaced by non-surgery treatment. However, many studies showed that surgery was benefit to patients who present with hemorrhage, perforation, or ileus [
19,
20], especially to PIL patients. PIL was not only difficult to make confirmed diagnosis but also likely to develop with complications, so it was usually needed surgical excision and then diagnosed by pathologic analysis. In our study, there were 41 PIL patients, which 38 patients accepted surgical treatment. We believed that surgery was the main treatment method of undetermined diagnosis of PGIL patients, with its irreplaceable advantages as follows: (1) surgery was an important means to gain the pathological diagnosis and determine diagnosis; (2) postoperative specimens could be graded and staged correctly in order to judge prognosis more exactly; (3) surgery might alleviate tumor load, relieve clinical symptoms, and strengthen effect of other treatment, such as postoperative chemotherapy; and (4) patients who were insensitive to other treatment or appeared life-threatening complications should choose surgery as soon as possible.
In summary, the reasonable clinical treatment method of PGIL should be made according to the location, clinical stage, pathologic pattern, and with complications or not.
Conclusions
In conclusion, our study showed that it was more common to find location of PGIL in the stomach than that in the intestine. The most common pathological type was MALT in the PGL group and DLBCL in the PIL group. The treatment of PGL was focused on chemotherapy. It was noting that since PIL was not only difficult to make confirmed diagnosis but also likely to develop with complications, so it was usually needed surgical excision and then diagnosed by pathologic analysis. Clinical stage and pathological pattern were related to prognosis of PGIL.
Consent
Written informed consent was obtained from the patient for the publication of this report and any accompanying images.
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Competing interests
The authors declare that they have no competing interests.
Authors’ contributions
All authors took part in the design of the study. ZG performed the statistical analyses, and all authors took part in the interpretation of the results. ZG drafted the manuscript, and ZL critically commented on the content as well as writing. All authors had read and approved the final version.