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Non-Hodgkin's lymphoma of the appendix and distal ileum. A rare case report
Aparna Govil Bhasker1, Jay D Kotecha2, Rahul Pandey3, Sachin Wani4
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 Submission||03-Jan-2021|
|Date of Decision||08-Apr-2021|
|Date of Acceptance||19-Apr-2021|
|Date of Web Publication||03-Jun-2021|
Aparna Govil Bhasker,
A 807, Adani Western Heights, JP Road, Opp Gurudwara, Manish Nagar, Andheri West, Mumbai - 400 053, Maharashtra
Source of Support: None, Conflict of Interest: None
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
| ¤ Introduction|| |
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. Lymphomas of the appendix are extremely rare and very few cases have been reported in the literature till date. 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|| |
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|| |
Malignant tumours of the small intestine are uncommon and constitute less than 3% of all GI tract malignancies. 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. 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. 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. They constitute 0.4% of all neoplasms of the GI tract. Majority of appendiceal tumours are carcinoids. The incidence of primary lymphoma of the appendix is about 0.015% of appendicectomy specimens. 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. 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. 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.
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]