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Non-Hodgkin's lymphoma of the appendix and distal ileum. A rare case report


1 Bariatric and Laparoscopic Surgery, Saifee Hospital, Mumbai, Maharashtra, India
2 Kotecha's Endoscopy Centre, Mumbai, Maharashtra, India
3 Consultant Pathologist, Accura Healthcare and Diagnostics, Mumbai, Maharashtra, India
4 Laparoscopic and GI Surgeon, Currae Specialty Hospital, Thane, Maharashtra, India

Date of Submission03-Jan-2021
Date of Decision08-Apr-2021
Date of Acceptance19-Apr-2021
Date of Web Publication03-Jun-2021

Correspondence Address:
Aparna Govil Bhasker,
A 807, Adani Western Heights, JP Road, Opp Gurudwara, Manish Nagar, Andheri West, Mumbai - 400 053, Maharashtra
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jmas.JMAS_3_21

  Abstract 

Primary gastrointestinal (GI) tract lymphomas constitute 1%–4% of all GI malignancies. Primary lymphomas of appendix are even more rare and are seen in 0.015% of all appendicectomy specimens. Here, we report a rare case of non-Hodgkin's lymphoma tumours in the appendix and distal ileum in a non-immune compromised young male patient who presented with intermittent intussusception and pain in the right iliac fossa. A laparoscopic right hemicolectomy was performed and the patient recovered uneventfully. Adjuvant chemotherapy (CHOP) in the form of CHOP regimen has been further advised.


Keywords: Laparoscopic right hemicolectomy, lymphoma of ileum, malignant tumour, non-Hodgkin's lymphoma, non-Hodgkin's lymphoma of the appendix, tumours of the ileum



How to cite this URL:
Bhasker AG, Kotecha JD, Pandey R, Wani S. Non-Hodgkin's lymphoma of the appendix and distal ileum. A rare case report. J Min Access Surg [Epub ahead of print] [cited 2021 Jun 14]. Available from: https://www.journalofmas.com/preprintarticle.asp?id=317732



  Introduction Top


Although the alimentary tract is the most common site of extranodal lymphomas, primary gastrointestinal tract (GI) lymphomas constitute only about 1%–4% of all GI malignancies.[1] Lymphomas of the appendix are extremely rare and very few cases have been reported in the literature till date.[2] Here, we report a rare case of primary B-cell lymphoma occurring together in the distal ileum and the appendix in a non-immune compromised young male patient. We could not find a similar case reported earlier in the literature and to our knowledge, this may be the first such case report.


  Case Report Top


A 38-year-old male patient presented with a history of intermittent pain in the right iliac fossa for 2 months which had increased in intensity and frequency for the last 15 days. There was no history of fever, vomiting, bowel or urinary complaints. An ultrasound of the abdomen was advised which revealed a mucocele of the appendix. On a high degree of suspicion, a computed tomography (CT) scan of the abdomen and pelvis was advised prior to preparing him for a laparoscopic appendicectomy. CT scan revealed an irregular enhancing mass lesion in the terminal ileum extending across the ileo-caecal junction (ICJ) along the medial wall of the caecum into the proximal two-third of the appendix without any frank extra-serosal extension. Other routine investigations revealed a haemoglobin of 13.1 g%, white blood cell count – 8300/mm3, seronegative for HIV, SGOT – 25 U/L, SGPT – 44 U/L, alkaline phosphatase – 156 U/L and serum creatinine – 0.9 mg/dL. High-resolution computed tomography of the chest was normal. Colonoscopy revealed a large polypoidal growth in terminal ileum, 7 cm proximal to the ICJ [Figure 1]. The growth was seen to be intussuscepting into the caecum. Surrounding mucosa appeared normal. Multiple biopsies taken revealed non-Hodgkin's lymphoma (NHL) of the B-cell type (non-germinal center B ['GCB'] like phenotype). Positron-emission tomography of the CT was performed. A total laparoscopic right hemicolectomy was performed with a side-to-side stapled ileo-colic anastomosis [Figure 2]. The specimen was retrieved through a 5 cm Pfannenstiel incision using a wound protector. The patient had an uneventful post-operative course and recovered well. He was discharged after 5 days from the hospital.
Figure 1: Colonoscopic picture of polypoidal growth in the distal ileum leading to intussusception

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Figure 2: Gross picture of the resected specimen after right hemicolectomy

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The final histopathology report revealed two tumours. First one, a polypoidal proliferative growth of 6 cm × 3.5 cm × 2.5 cm involving the terminal ileum 7 cm proximal to the colon. It involved the bowel wall partly. The tumour was invading the wall of the colon up to the pericolic adipose tissue. Both the proximal and distal resection margins were clear. The second one was in the appendix measuring 6.5 cm × 3.2 cm × 2.8 cm. Tips of the appendix were free of the tumour and both the tumours were separated by an island of normal mucosa in between. Both the tumours were diffuse large B-cell lymphoma non-GCB type. Serosa was free of tumour. Twenty-two resected lymph nodes were negative for tumour infiltration. Medical oncology opinion was taken and adjuvant chemotherapy (CHOP regimen) has been further planned.


  Discussion Top


Malignant tumours of the small intestine are uncommon and constitute less than 3% of all GI tract malignancies.[3] Twelve per cent of these are constituted by lymphomas, others being adenocarcinomas, carcinoid tumours and GI sarcomas. The most common site for lymphoma in GI tract is the stomach (60%–75%) followed by the distal small bowel and ileum.[4] The implicated risk factors are Helicobacter pylori infection, Campylobacter jejuni colonisation in small intestine, HIV, other immune-deficiency disorders, patients with solid organ transplant and inflammatory diseases of the small intestine.[5] Due to non-specific symptoms, diagnosis may be delayed and patients may present with complications such as pain, GI bleed, obstruction or perforation. In our patient, the repeated intermittent episodes of pain could be explained by intermittent intussusception due to the polypoidal growth in the distal ileum. In these cases, surgical resection followed by adjuvant CHOP is the treatment of choice. Radiation therapy is not used due to high risk of post-radiation enteritis. Most common CHOP regimen used is cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP). In some cases, rituximab is added, and it is then called as R-CHOP regime.

Malignant tumours of the appendix are even more rare and are seen in only about 1% of all appendicectomies.[6] They constitute 0.4% of all neoplasms of the GI tract.[7] Majority of appendiceal tumours are carcinoids. The incidence of primary lymphoma of the appendix is about 0.015% of appendicectomy specimens.[8] These are almost always NHLs and are more common in men. Earlier these were seldom diagnosed prior to surgery. However, over the last few decades, the incidence of GI lymphomas has been on the rise. Coupled with increasing imaging, these are being detected prior to surgery as well.[9] They may present with symptoms like acute appendicitis due to obstruction of the appendiceal lumen because of the tumour or with more insidious symptoms spread over a few months. Literature about appendiceal lymphomas is limited to a few case reports, thus emphasising on its rarity.[10] CHOP in the form of CHOP or R-CHOP regimen is the mainstay of treatment. However, surgery may be needed in some cases.

In our patient, we had two NHL lesions, one in the distal ileum and the other in the appendix. On histopathological examination, it was seen that these were separated by an island of normal mucosa in between. However, it is difficult to decipher if these lesions could be classified as multiple primary tumours or if they were a part of the same disease process. Despite an extensive search of the literature, we could not find a similar case, and this is probably the first such case being reported where two NHL lesions were found together in the ileum and the appendix.

Ethical statement

The patient's identity has not been revealed in the above case report. As this is a case report, ethics committee approval is not applicable. No animals were harmed during this study.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Howell JM, Auer-Grzesiak I, Zhang J, Andrews CN, Stewart D, Urbanski SJ. Increasing incidence rates, distribution and histological characteristics of primary gastrointestinal non-Hodgkin lymphoma in a North American population. Can J Gastroenterol 2012;26:452-6.  Back to cited text no. 1
    
2.
Fu TY, Wang JS, Tseng HH. Primary appendiceal lymphoma presenting as perforated acute appendicitis. J Chin Med Assoc 2004;67:629-32.  Back to cited text no. 2
    
3.
Naef M, Bühlmann M, Baer HU. Small bowel tumors: diagnosis, therapy and prognostic factors. Langenbecks Arch Surg 1999;384:176-80.  Back to cited text no. 3
    
4.
Ghimire P, Wu GY, Zhu L. Primary gastrointestinal lymphoma. World J Gastroenterol 2011;17:697-707.  Back to cited text no. 4
    
5.
Andrews CN, John Gill M, Urbanski SJ, Stewart D, Perini R, Beck P. Changing epidemiology and risk factors for gastrointestinal non-Hodgkin's lymphoma in a North American population: population-based study. Am J Gastroenterol 2008;103:1762-9.  Back to cited text no. 5
    
6.
McCusker ME, Coté TR, Clegg LX, Sobin LH. Primary malignant neoplasms of the appendix: A population-based study from the surveillance, epidemiology and end-results program, 1973-1998. Cancer 2002;94:3307-12.  Back to cited text no. 6
    
7.
Connor SJ, Hanna GB, Frizelle FA. Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7,970 appendectomies. Dis Colon Rectum 1998;41:75-80.  Back to cited text no. 7
    
8.
Guo J, Wu G, Chen X, Li X. Primary appendiceal lymphoma presenting as suspected perforated acute appendicitis: Clinical, sonography and CT findings with pathologic correlation. Int J Clin Exp Pathol 2014;7:7068-71.  Back to cited text no. 8
    
9.
Crump M, Gospodarowicz M, Shepherd FA. Lymphoma of the gastrointestinal tract. Semin Oncol 1999;26:324-37.  Back to cited text no. 9
    
10.
Collins DC. 71,000 human appendix specimens. A final report, summarizing forty years' study. Am J Proctol 1963;14:265-81.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]



 

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2004 Journal of Minimal Access Surgery
Published by Wolters Kluwer - Medknow
Online since 15th August '04