Indian Journal of Pathology and Microbiology

: 2008  |  Volume : 51  |  Issue : 3  |  Page : 392--394

Primary marginal zone B-cell lymphoma of appendix

S Radha, Tameem Afroz, G Satyanarayana 
 Department of Pathology and General Surgery, Kamineni Hospitals, L.B. Nagar, Hyderabad, India

Correspondence Address:
S Radha
Plot No. 20, Road No.1, Alakapuri, Hyderabad - 500 035 Andhra Pradesh


Primary lymphomas of appendix are extremely rare tumors. The first case of primary lymphoma of appendix was reported by Warren in the year 1898. Incidence of primary lymphoma of appendix is 0.015% of all gastrointestinal lymphomas. This is a report of primary marginal zone B-cell lymphoma of appendix which presented as appendicular mass. As some cases are incidentally discovered, this case emphasizes that histological examination of all appendicectomy specimens is mandatory.

How to cite this article:
Radha S, Afroz T, Satyanarayana G. Primary marginal zone B-cell lymphoma of appendix.Indian J Pathol Microbiol 2008;51:392-394

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Radha S, Afroz T, Satyanarayana G. Primary marginal zone B-cell lymphoma of appendix. Indian J Pathol Microbiol [serial online] 2008 [cited 2021 Dec 2 ];51:392-394
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Gastrointestinal tract is the most common site of extranodal lymphomas and accounts for 30-45% of all extranodal cases. Stomach is the most commonly involved organ followed by small intestine, colon and esophagus. The incidence of primary lymphoma of appendix is 0.015% of all gastrointestinal lymphomas. We report a case of primary marginal zone B-cell lymphoma of appendix presenting as appendicular mass. [1] The appendicular mass was excised along with a segment of ileum and caecum. Total body PET scan and bone marrow aspiration were done as staging procedures and the patient was initiated on chemotherapy.

 Case history

A 61-year-old male presented to his personal physician with pain right iliac fossa of 3 days' duration. Pain was associated with nausea and low-grade fever. Pain was localized. There was no history of loss of weight or appetite. There was no history of lower gastrointestinal bleed. There were no urinary symptoms. There is no history of diabetes or hypertension. He gave history of recurrent episodes of similar pain during the past 3 months. There was a history of constipation of 2 months' duration He gave history of laparoscopic hernia repair 4 years back. He was put on antibiotic Augment in 625mg, thrice a day and was referred for further management to our hospital.

On examination, there was rebound tenderness in the right iliac fossa, abdomen was soft and a mass of 10 6.0 cm was palpable in the right iliac fossa, margins of which were ill-defined. It was firm in consistency and tender. Liver and spleen were not palpable. There was no peripheral lymphadenopathy. His routine hematological and biochemical parameters were within normal limits.

CT abdomen obtained with oral and intravenous contrast revealed dilated and enlarged appendix with thickened walls. There was fat stranding in the adjacent mesentery associated with thickening of the Gerotas and lateroconal fascia. With a provisional diagnosis of appendicular mass, the patient was taken up for diagnostic laparoscopy. Intraoperatively, appendix was grossly enlarged with dense adhesions to caecum. Bowel was adherent to the lateral abdominal wall. There were no lymphnodes identified and there was no free fluid. Rest of the bowel appeared normal. Hand-assisted laparoscopic ileocaecal resection was performed.

Grossly, the specimen consisted of a segment of intestine measuring 14 cm. Appendix was enlarged and measured 8.0 3.0 cm [Figure 1]. On cut section, the wall of the appendix was grossly thickened with irregular nodular gray-white areas. Lumen was compressed. The nodular areas were hard and extended into the periappendiceal fat. Rest of the intestinal mucosa and wall appeared normal.

Microscopic examination: There was ulceration of mucosa of appendix and the wall revealed lymphoid cells diffusely arranged. Cells were small with scant cytoplasm [Figure 2]. Nuclei had finely clumped chromatin. Mitoses were sparse. Few karyorrhectic areas were seen. These cells were seen extending into the serosal fat. These were immunoreactive to CD20 [Figure 3] and negative for CD3, CD5 and CD23. MIB-1 expression was 20-30% over most areas. Histological and immunoprofile findings were consistent with a marginal zone B-cell lymphoma. Uninvolved appendix revealed reactive follicular hyperplasia. Sections of ileum and caecum did not reveal any specific pathology.

As a staging work-up, a total-body PET scan was done, which was within normal limits. His bone marrow aspiration did not reveal infiltration.

He was initiated on chemotherapy with R-CHOP regimen. The patient was advised six cycles of the above regimen. The patient is symptom-free and on regular follow-up.


The incidence of primary lymphomas of appendix is estimated as 0.015% of all gastrointestinal lymphomas. In our institute, in over 10 years (1997-2006), 1060 appendicectomy specimens were analyzed. This was the only case of Non Hodgkins lymphoma reported. Lymphomas of appendix usually present in the second and the third decades of life. [2] Our patient was older than the age group described in the literature. These usually present as acute appendicitis. [3] In a review of 46 cases reported, 31 presented with acute or subacute pain and five were incidentally discovered. [4] Other symptoms described are anorexia, weight loss, nausea and vomiting. Right lower quadrant mass may be palpable on examination. Rarely intussception or lower gastrointestinal bleed may be present. [5] Appendiceal lymphomas are often diagnosed postoperatively as the clinical findings are nonspecific, leading to a delay in diagnosis.

The Armed Forces Institute of Pathology reported five cases of Non Hodgkins lymphoma of appendix, where the diameter of appendix varied from 2.5-4.0 cm and length varied from 7-17 cm. In the present case, the diameter of appendix was 3.0 cm and length was 8.0 cm. Inflamed appendix without neoplasm rarely measured more than 1.5 cm in diameter.

Gastrointestinal tract is the most frequently involved extranodal site of Non Hodgkins lymphoma and account for 30-45% of extranodal cases and 4-20% of all Non Hodgkins lymphoma. [6] In a series of 29 cases reported in the last 3 decades, nine cases were Burkitts lymphoma, two were mantle cell lymphomas, three were T-cell lymphomas, four were B-cell diffuse large cell lymphomas and one was B-cell marginal zone lymphoma. [7] Extranodal lymphomas are common in patients with immunocompromised states like AIDS and in organ transplant recipients. In these populations, extranodal lymphomas account for more than 80% of abdominal lymphomas. [8]

The present case in discussion is a primary marginal zone B-cell lymphoma, which is the rarest of all the cases described in the literature.

In conclusion, we describe a rare case of primary marginal zone B-cell lymphoma of appendix in an elderly patient who presented with subacute pain and appendicular mass. Incidence of primary lymphomas of appendix in our institute was 0.01%, which correlates with that described in the literature.


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