|Year : 2017 | Volume
| Issue : 10 | Page : 57-60
Primary B-cell jejunal maltoma in a young adult male: A case report and review of literature
Ifeoma Florence Ezejiofor1, Christian Chika Ogbu1, Michael Emeka Onwukamuche1, Chinedu Onwuka Ndukwe1, Kingsley Chike Madubuike1, Felix Emeka Menkiti1, Nnamdi Sergius Ozor1, Emmanuel Chinonso Okakpu2
1 Department of Histopathology, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra State, Nigeria
2 Department of Haematology and Blood Transfusion, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra State, Nigeria
|Date of Web Publication||13-Feb-2018|
Ifeoma Florence Ezejiofor
Department of Histopathology, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra State
Source of Support: None, Conflict of Interest: None
Primary jejunal lymphoma of mucosa-associated lymphoid tissue type is a relatively uncommon extranodal marginal zone lymphoma of nongastric low-grade B-cell non-Hodgkin's neoplasm that arises in the cells of mucosal tissue of the small intestine. The aim of this article is to present Stage IIE (T4N0MX) primary jejunal maltoma with multiple exophytic lymphomatous polyposis in a young adult male of 27 years with metastasis to the left adrenal gland. This article also highlights the association of this disease with low socioeconomic status and the use of immunohistochemical stains to aid in diagnosis.
Keywords: Adrenal metastasis, low socioeconomic status, primary jejunum maltoma, young adult male
|How to cite this article:|
Ezejiofor IF, Ogbu CC, Onwukamuche ME, Ndukwe CO, Madubuike KC, Menkiti FE, Ozor NS, Okakpu EC. Primary B-cell jejunal maltoma in a young adult male: A case report and review of literature. N Niger J Clin Res 2017;6:57-60
|How to cite this URL:|
Ezejiofor IF, Ogbu CC, Onwukamuche ME, Ndukwe CO, Madubuike KC, Menkiti FE, Ozor NS, Okakpu EC. Primary B-cell jejunal maltoma in a young adult male: A case report and review of literature. N Niger J Clin Res [serial online] 2017 [cited 2022 Jan 27];6:57-60. Available from: https://www.mdcan-uath.org/text.asp?2017/6/10/57/225335
| Introduction|| |
Gastrointestinal tract (GIT) is the most common extranodal site involved by lymphoma, accounting for 5%–20% of all cases. GIT lymphomas are most frequently located in the stomach followed by small intestine and ileocecal region. Primary GIT lymphoma is, however, very rare as compared with secondary GIT lymphoma constituting only 1%–4% of all GIT malignancies. Primary small intestinal malignant tumors are equally very rare, constituting <2% of all tumors of GIT, of which 15%–20% are primary small intestinal lymphoma. These small intestinal lymphomas are more heterogeneous and include marginal zone mucosa-associated lymphoid tissue (MALT) lymphoma, diffuse large B-cell lymphoma (DLBCL), enteropathy-associated T-cell lymphoma, mantle cell lymphoma, follicular lymphoma, and immunoproliferative small intestinal disease (IPSID) also known as alpha chain disease, which is a MALT-associated lymphoma due to Campylobacter jejuni infection. Of all these lymphomas, DLBCL is the most common in essentially all sites and most aggressive, while MALT type is the most common indolent subtype and represents 7% of all non-Hodgkin's lymphomas in the Western world. Most MALT lymphomas of the small intestine occur in the ileum (60%–65%) followed by jejunum (20%–25%), while duodenum and other sites account for 6%–8% and 8%–9%, respectively. There is no established relationship between extranodal marginal zone B-cell lymphoma (MALT type) of the small intestine with Helicobacter pylori organism as it was in the stomach, but reports with Helicobacter heilmannii infection exist.
| Case Report|| |
A 27-year-old secondary school dropout, an apprentice in trading, first in the family of 7 children whose parent are low salary earners presented at the accident and emergency with complaints of loin pain of 2 months duration. Physical examination revealed a blood pressure of 150/90 mmHg, epigastric tenderness, severe left lumbar tenderness, left renal angle tenderness, left Grade III varicocele, and right Grade I varicocele. He does not smoke, and retroviral screening was negative.
Pre- and post-surgery fasting blood sugar, random blood sugar, and E/U/Cr are within normal ranges. The white blood cell count is also within normal range; presurgery value is 7.28 × 109/ L, while postsurgery value is 5.33 × 109/L.
Ultrasound scan showed multiple masses in the splenic hilum. Similar masses were seen in the left renal hilum. Computed tomography scan showed a well-defined roun/ded nonenhancing mixed density mass in the left perinephric space. It measured 7.6 cm × 6.9 cm × 5.8 cm and not arising from the kidney. Intravenous urogram done also showed no functional relationship between the mass and the kidney.
He underwent exploratory laparotomy. Intraoperative findings were a highly vascularized left pararenal tumor, abutting the left renal pelvis and appeared encapsulated. Multiple jejunal tumors (annular) >5 in number involving about 105 cm length of jejunum with normal intervening loop were also seen.
The specimen was sent for histology and immunohistochemistry. He was discharged after 12 days on admission and subsequently started on chemotherapy (cyclophosphamide, vincristine, doxorubicin, and prednisolone). He has had two courses of chemotherapy and currently doing well.
At the histopathology department, a tubular segment of intestinal tissue measuring 79 cm in length and 4 cm in widest diameter was received. Cut section showed multiple exophytic tumors ranging in size from 1 cm × 1 cm to 4 cm × 4 cm with fish flesh cut surfaces [Figure 1]. Thirty-three lymph nodes were identified ranging in size from 0.5 cm × 1 cm to 1.5 cm × 2 cm with greenish-white cut sections. There is an accompanying nodular firm tissue measuring 8 cm × 7 cm × 2 cm and weighs 100 g with variegated appearance on cut sections.
|Figure 1: Multiple exophytic tumors within the jejunal mucosa exhibiting fish flesh appearance on cut sections|
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Histology showed a malignant lymphoid neoplasm composed of atypical small lymphocytes having condensed chromatin pattern and scanty cytoplasm with prominent nucleoli. These neoplastic lymphocytes are disposed in sheets and infiltrating the mucosae (lymphoepithelial lesion) [Figure 2]a and [Figure 2]b higher magnification ×400], the muscularis propria, and serosa in areas. Few abnormal mitotic figures were present. The thirty-three harvested lymph nodes and both resection margins are free of tumor invasions. However, the perinephric mass (left adrenal) and omentum [Figure 3] are involved by tumor of similar morphology. Immunohistochemistry showed strong positivity for CD20 and moderate positivity for CD79a [Figure 4]a and [Figure 4]b but negative stains on CD3, CD5, CD23, and Bcl-2. The proliferative index as measured by Ki67 is <5%. Overall features are those of a non-Hodgkin's lymphoma: extranodal marginal zone lymphoma.
|Figure 2: (a) Sheets of malignant lymphocytes invading the mucosae, thereby giving rise to a lymphoepithelial lesion. (b) Sheets of malignant lymphocytes invading the mucosae at higher magnification ×400|
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|Figure 4: (a) The tumor is moderate positivity for CD79a (×40 obj). (b) Sheets of malignant lymphocytes invading the mucosae at higher magnification ×400|
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| Discussion|| |
MALT lymphoma of small intestine occurs in all ethnic groups, races, and the world at large but few cases have been reported in Nigeria. Primary small intestine B-cell lymphomas are uncommon in North America, European, and Asian countries although DLBCL subtype seems to be the most common lymphoma in those areas, while IPSID subtype occurs in the Middle East and Mediterranean areas. Some authors have also reported IPSID to occur in underprivileged young adult in part of South America, Nigeria, and cape region of South Africa.
In Lagos, Abdulkareem et al. analyzed 713 cases of GIT tumors; only four cases of non-Hodgkin's lymphoma of small intestine were reported. In Sokoto, Chinaka et al. analyzed 240 intestinal biopsies; only two cases of malignant tumors of small intestine were seen; one non-Hodgkin lymphoma in a 3-year-old child and one MALT lymphoma in a 50-year-old male. In River state Nigeria, Udoye et al. reported a case of colonic maltoma of caecum, ascending colon, and transverse colon in a 40-year-old male. Research on maltoma of small intestine in Nigeria is very few, and most cases were present in colon and stomach, no case of jejunal maltoma was seen in south-east Nigeria. The index case showed jejunual location of this tumor which is first reported a case in South East Nigeria.
Many studies have shown a slight male preponderance and an association with lower socioeconomic status. Studies also show that age of presentation varies with the histologic subtype of the lymphoma with DLBCL occurring in increasing age and peaks at the seventh decade of life, while maltoma of the IPSIP affects older children and younger adults. The index case occurred in a young adult male of 27 years old with low socioeconomic background, an apprentice in trading, whose parents are low salary earners.
The small intestinal lymphomas usually present as a polyp or ulcerating mass, an intramural lesion, or stricture. In contrast, involvement of the GIT in the form of multiple polyploidy lesions is rare. The index case showed a morphologic appearance of multiple exophytic lymphomatous polyposis in the entire length of the jejunum with a clinical feature of acute abdomen. Although the clinical presentation is nonspecific, the most common presentation is in the form of acute abdomen which was seen in this patient.
At the time of diagnosis low grade MALT lymphoma are usually at stage IE or IIE and dissemination is slow.
The mesenteric lymph nodes are usually involved (Stage IIE) but the dissemination outside the abdomen is unusual at the time of diagnosis. The index case showed similarity in staging appearance with tumor invasion in the retroperitoneal organ (left adrenal) without the involvement of 33 harvested lymph nodes classifying the cases as T4N0Mx Stage 2E.
The hallmark of diagnosis are histology, immunohistochemistry, and molecular study. The histologic features of low grade B-cell lymphoma of MALT types are similar irrespective of site of origin. Currently, there are no specific markers that can distinguish MALT lymphoma from other low-grade B-cell neoplasms. However, MALT lymphoma of small intestine does not express CD5, CD3, CD23, CD10, Bcl-2, and cyclin D1, but they show expression of CD20 and CD79a., The index case expressed strong positivity for CD20 and moderate positivity for CD79a [Figure 4]a and [Figure 4]b but negative stains on CD3, CD5, CD23, and Bcl-2. The proliferative index as measured by Ki67 is <5%.
Although there is no universal accepted guideline for the treatment of GIT MALT lymphomas, protocol treatment depends on the extent of the disease and stage of the tumor. Stage I–II involves surgery or targeted radiotherapy with systemic antibiotics directed against etiopathogenic microorganism and conventional chemotherapy. The index case had surgery, antibiotic coverage with chemotherapy, and currently on follow-up.
Prognosis for extranodal marginal zone lymphoma (MALT type) is excellent with a 5-year survival rate of >90% and 10-year survival rate of 75%–80% but maltoma of the intestine is worse than gastric type with a 5-year survival rate of 44%–75%.
| Conclusion|| |
Jejunal maltoma with multiple lymphomatous polyposis in a young adult is a relatively uncommon diagnosis in our environment. This report has highlighted a rare morphologic presentation and low socioeconomic status as likely associated risk factor.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]