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  Table of Contents  
Year : 2014  |  Volume : 51  |  Issue : 3  |  Page : 306-308

Primary Intestinal Lymphoma: A Clinicopathological Study

1 Department of Pathology, Kannur Medical College, India
2 Basaveshwar Medical College, Chitraduraga, Karnataka, India
3 Department of Pathology, Pariyaram Medical College, Kannur, Kerala, India

Date of Web Publication10-Dec-2014

Correspondence Address:
P J Yaranal
Department of Pathology, Kannur Medical College
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-509X.146791

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 » Abstract 

Introduction: Primary intestinal lymphoma (PIL) is defined as an extranodal lymphoma arising in the intestine. This study includes 11 PILs. Objectives: The objective of this study was to evaluate the clinicopathological characteristics of primary non-Hodgkin's lymphoma (NHL) in the small and large intestine. All cases were reclassified according to the World Health Organization classification of lymphoma in 2001. Immunohistochemistry (IHC) was used to confirm the histopathological diagnosis. Materials and Methods: Eleven cases of primary non-Hodgkin in the small and large intestine were studied retrospectively in a 5 year period. There were seven cases of resected intestinal specimens and four biopsy specimens. Five cases from ileum and two cases each from caecum and duodenum and one case each from jejunum and duodenum. Hematoxylin and eosin stained sections were studied with light microscopy and IHC for CD5, 20, 21 and cyclin D1 were done. Results: It is a retrospective study of 11 cases of intestinal lymphomas. Abdominal pain and abdominal lump were two main common presenting symptoms. NHLs were more common in the small intestine. PILs are common in adults (75% above 46 years) with male preponderance. All were NHLs and B-cell type. Five cases were diffuse large B-cell lymphoma and four cases each were mucosa-associated lymphatic tissue lymphoma and follicular lymphoma. One case was lymphomatoid polyposis or mantle cell lymphoma. Conclusion: PILs is rare and differs significantly from their gastric counterpart, not only in pathology but also with regard to clinical features, management and prognosis. Due to the lack of characteristic symptoms and a low incidence rate, PIL is misdiagnosed until serious complications occur, such as perforation and bleeding and hence needs to be accurately diagnosed.

Keywords: Intestine, lymphoma, non-Hodgkin′s lymphoma

How to cite this article:
Yaranal P J, Harish S G, Purushotham B. Primary Intestinal Lymphoma: A Clinicopathological Study. Indian J Cancer 2014;51:306-8

How to cite this URL:
Yaranal P J, Harish S G, Purushotham B. Primary Intestinal Lymphoma: A Clinicopathological Study. Indian J Cancer [serial online] 2014 [cited 2020 Nov 29];51:306-8. Available from:

 » Introduction Top

In the recent years, the incidence of non-Hodgkin's lymphoma (NHL) has increased worldwide, especially for primary extranodal lymphoma. Gastrointestinal (GI) tract accounts for 20-40% of all NHLs and is the most common extranodal site of presentation. Primary GI lymphomas constitute about 5.6% of total gut neoplasms. Almost half of the primary GI lymphomas are in the stomach accounts for 50-60%, followed by small intestine lymphoma 14-38% and 10-20% in the large intestine. Lymphomas originate from mucosa-associated lymphoid tissue (MALToma) of GI tract. The involvement of small and large bowel as the primary site is all the more rare. [1],[2],[3],[4],[5] Primary intestinal lymphoma (PILs) differ from gastric ones not only in pathology, but also as regards their clinical features, treatment and prognosis.

Histopathologically, 80-90% of primary GI lymphomas are of B-cell origin. Intestinal B-cell lymphomas World Health Organization (WHO) include MALT lymphoma, immunoproliferative small intestinal disease, mantle cell lymphoma, Burkitt lymphoma and diffuse large B-cell lymphoma (DLBCL). Intestinal T-cell lymphomas WHO include T-cell lymphoma and enteropathy associated T-cell lymphoma. [6] In terms of T-cell lymphoma, there is a discrepancy in the different sites of GI tract involved, i.e. the incidence of T-cell lymphoma increases with the site from the stomach to colon getting lower and this discrepancy is more significant among eastern populations. Helicobacter pylori infection, immunosuppression after solid organ transplantation, celiac disease, inflammatory bowel disease and human immunodeficiency virus infection may be the risk factors for GI lymphoma. [7]

Due to the lack of characteristic symptoms and a low incidence rate, PIL is misdiagnosed until serious complications occur, such as perforation or bleeding. This disease is of interest to pathologists and clinicians in part because of the treatable nature of disease in the initial stage, for a cure and physicians ability to relieve symptoms predictably. Aim of this retrospective study was to analyze the clinical features and anatomic and histological distribution of PILs and review of the literature.

 » Materials and Methods Top

We carried out this study to evaluate the prevalence and clinicopathological features of PILs and to compare our findings with published literature. We carried out a retrospective analysis of the records of histologically diagnosed cases of intestinal lymphomas over a 5 year period, from 2006 to 2011. Detail clinical history of the patients was taken from medical records. Clinical examination, hematological, biochemical and radiological investigations were available from records. Out of 11 cases, seven were resected specimens and four were endoscopic biopsy specimens.

Histopathological examination and immunophenotyping on the tissue sections were carried out and final diagnosis was made.

 » Results Top

During the 5 year study period, 11 cases of PIL were retrieved. There were five cases of lymphoma of ileum, two cases each of lymphoma of jejunum and caecum and one case each of lymphoma of the duodenum and rectum.

Age range was from 16 to 70 years and male to female ratio was 4.5:1. The most common presenting symptoms were abdominal pain (100%), abdominal mass (50%), followed by nausea, vomiting and weight loss (25%), fever (25%) and GI bleeding (15%) as shown in the [Table 1].
Table 1: Clinical features

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Table 2: Histopathological subtypes of intestinal lymphoma

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Table 3: Immunotyping

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[Table 2] shows histopathological subtypes of PILs. DLBCL [Figure 1] was most common variant accounting for 36%, followed by MALT lymphomas [Figure 2] and follicular lymphomas [Figure 3] variants accounts for 27% each and mantle cell lymphoma [Figure 4] constitutes for 10%. Immunophenotyping showed all cases to be of B-cell origin [Table 3].
Figure 1: Diffuse large B-cell lymphoma: (a) Confluent collections of large cells indicate a large cell lymphoma (H and E, ×5) and (b) immunohistochemistry for CD20 shows cytoplasmic staining of B-cells

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Figure 2: MALT lymphoma of caecum: (a) Lymphoid infiltrate extends deeper into the lamina propria (H and E, ×20). (b) Immunohistochemistry for CD21 shows many B-cells

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Figure 3: Follicular lymphoma: (a) Follicular architecture is variably prominent on H and E staining. (b) Immunohistochemical stains show the follicle centres are positive for CD20

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Figure 4: Mantle cell lymphoma: (a, b) Involvement by mantle cell lymphoma is often multifocal and may be subtle as in this case or produce the better known lymphomatoid polyposis; (c, d) Nuclear staining of variable intensity with Cyclin D1 is characteristic with focal CD5 staining

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 » Discussion Top

A total 11 cases of PIL were encountered during 5 year period. Of this eight were small intestinal and three were large intestinal lymphomas. PIL is a male predominant disease and male to female ratio was 4.5:1 in our study. The mean age at presentation in our study was (43 years). These observations were similar to the Wang et al. [8] and Li et al. [9] studies, but in other studies most common in old age. [10],[11],[12]

The most common presenting symptom in our series were abdominal pain, abdominal mass followed by anorexia and weight loss. This is similar to that observed in western series. [8],[9],[10],[11],[12],[13],[14],[15]

The ileocecal region was the most common site with a frequency of 63.5%, which is consistent with Wang et al., [8] Li et al., [9] Wang et al. [14] and Kohno et al. [15] studies but in other studies colorectal involvement was most common. [10],[11],[12],[13] Probably, due to relatively higher proportion of lymphoid tissue in these areas or because of lack of precise definition of the ileocecal region which was missing in most of the reports. Moreover, higher ileocecal region involvement rate could be seen in western populations [10],[16] compared with that of eastern ones. [15] This discrepancy is similar to the geographic difference of colon carcinoma between the West and East. Diet habit, which acts as a kind of exposure factor for colon malignancies, may be attributable to this, although no such a correlation has been established in lymphoma.

High grade B-cell lymphoma was the most common subtype in all the studies. DLBCL (36%) and MALT and follicular lymphoma (27%) were most common histopathological subtypes in the present study, which is similar to the other reports. [8],[9],[10],[11],[12],[13],[14],[15]

Many of studies have shown that intestinal lymphoma had poorer survival than gastric lymphoma, [1],[10] probably due to a higher proportion of aggressive lymphoma, such as DLBCL and T-cell lymphoma seen in this area. [10],[11],[12],[13],[14],[15] However, no studies have found survival difference between NHL patients with small intestinal and large intestinal involvement. [1],[8]

 » Conclusion Top

PIL is a heterogeneous disease entity. High grade tumors are more common than the low grade tumors. However, a large-scale prospective investigation of PIL is difficult due to its low incidence and complicated histological subtypes and hence need for further multicenter prospective study to create a database large enough for definitive analysis to rationalize the treatment of such patients.

 » References Top

Nakamura S, Matsumoto T, Iida M, Yao T, Tsuneyoshi M. Primary gastrointestinal lymphoma in Japan: A clinicopathologic analysis of 455 patients with special reference to its time trends. Cancer 2003;97:2462-73.  Back to cited text no. 1
Yoshino T, Miyake K, Ichimura K, Mannami T, Ohara N, Hamazaki S, et al. Increased incidence of follicular lymphoma in the duodenum. Am J Surg Pathol 2000;24:688-93.  Back to cited text no. 2
Zucca E, Roggero E, Bertoni F, Cavalli F. Primary extranodal non-Hodgkin's lymphomas. Part 1: Gastrointestinal, cutaneous and genitourinary lymphomas. Ann Oncol 1997;8:727-37.  Back to cited text no. 3
Amer MH, el-Akkad S. Gastrointestinal lymphoma in adults: Clinical features and management of 300 cases. Gastroenterology 1994;106:846-58.  Back to cited text no. 4
Wong MT, Eu KW. Primary colorectal lymphomas. Colorectal Dis 2006;8:586-91.  Back to cited text no. 5
Zinzani PL, Frezza G, Bendandi M, Barbieri E, Gherlinzoni F, Neri S, et al. Primary gastric lymphoma: A clinical and therapeutic evaluation of 82 patients. Leuk Lymphoma 1995;19:461-6.  Back to cited text no. 6
Crump M, Gospodarowicz M, Shepherd FA. Lymphoma of the gastrointestinal tract. Semin Oncol 1999;26:324-37.  Back to cited text no. 7
Wang GB, Xu GL, Luo GY, Shan HB, Li Y, Gao XY, et al. Primary intestinal non-Hodgkin's lymphoma: A clinicopathologic analysis of 81 patients. World J Gastroenterol 2011;17:4625-31.  Back to cited text no. 8
Li B, Shi YK, He XH, Zou SM, Zhou SY, Dong M, et al. Primary non-Hodgkin lymphomas in the small and large intestine: Clinicopathological characteristics and management of 40 patients. Int J Hematol 2008;87:375-81.  Back to cited text no. 9
Koch P, del Valle F, Berdel WE, Willich NA, Reers B, Hiddemann W, et al. Primary gastrointestinal non-Hodgkin's lymphoma: I. Anatomic and histologic distribution, clinical features, and survival data of 371 patients registered in the German multicenter study GIT NHL 01/92. J Clin Oncol 2001;19:3861-73.  Back to cited text no. 10
Gurney KA, Cartwright RA, Gilman EA. Descriptive epidemiology of gastrointestinal non-Hodgkin's lymphoma in a population-based registry. Br J Cancer 1999;79:1929-34.  Back to cited text no. 11
Shukla K, Patel T, Shukla J, Palanki S. Primary gastrointestinal lymphoma - A clinicopathologic study. Indian J Pathol Microbiol 2007;50:296-9.  Back to cited text no. 12
Bai CM, Yang T, Xü Y, Zhang W, Liu XL, Zhu YL, et al. Clinical analysis of 32 primary intestinal non-Hodgkin's lymphoma. Zhonghua Zhong Liu Za Zhi 2006;28:142-4.  Back to cited text no. 13
Wang SL, Liao ZX, Liu XF, Yu ZH, Gu DZ, Qian TN, et al. Primary early-stage intestinal and colonic non-Hodgkin's lymphoma: Clinical features, management, and outcome of 37 patients. World J Gastroenterol 2005;11:5905-9.  Back to cited text no. 14
Kohno S, Ohshima K, Yoneda S, Kodama T, Shirakusa T, Kikuchi M. Clinicopathological analysis of 143 primary malignant lymphomas in the small and large intestines based on the new WHO classification. Histopathology 2003;43:135-43.  Back to cited text no. 15
Bairey O, Ruchlemer R, Shpilberg O. Non-Hodgkin's lymphomas of the colon. Isr Med Assoc J 2006;8:832-5.  Back to cited text no. 16


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1], [Table 2], [Table 3]

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