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Extranodal Marginal Zone Lymphoma Presenting within the Meckel Diverticulum as Diverticulitis: A Case Report
A Nael1, M L Wu2, P Nagesh Rao3
1Department of Pathology and Laboratory Medicine, University of California Irvine Medical Center, 101 The City Drive, Orange, CA 92868, USA.
Abstract:
Meckel diverticulum is the most common congenital defect of the gastrointestinal tract. It can be asymptomatic or mimic appendicitis and may be complicated by bleeding, diverticulitis, obstruction, and, rarely, neoplasia. We report the first case of extranodal marginal zone lymphoma occupying a Meckel diverticulum. A 44-year-old man with history of colonic diverticulitis presented to the emergency department for evaluation of acute abdominal pain. Radiography showed enteric obstruction, prompting diagnostic laparoscopy. Above the level of mid-ileum an intact Meckel diverticulum was identified. Microscopy showed extensive infiltration of sheets of small lymphocytes with abundant cytoplasm (monocytoid B-cells) prominently in submucosa and focally transmural involving serosal adipose tissue with multiple reactive germinal centers. The immunostains showed positivity for CD20, BCL-2, and CD43 (weak) and negativity for CD3, CD5, BCL-1, CD10, and BCL-6 in monocytoid B-cells. Fluorescence in situ hybridization studies revealed API2-MALT1 fusion signals consistent with t(11;18)(q21;q21), which confirmed the diagnosis of extranodal marginal zone lymphoma, also known as mucosa associated lymphoid tissue lymphoma.
Insights
This case report details the first instance of extranodal marginal zone lymphoma within a Meckel diverticulum. This rare gastrointestinal lymphoma, also known as MALT lymphoma, was identified in a patient presenting with acute abdominal pain.
Area of Science:
- Gastroenterology
- Oncology
- Pathology
Background:
- Meckel diverticulum is the most common congenital gastrointestinal anomaly.
- It can present asymptomatically or mimic other conditions like appendicitis.
- Complications include bleeding, diverticulitis, obstruction, and rarely, neoplasia.
Purpose of the Study:
- To report the first documented case of extranodal marginal zone lymphoma (EMZL) arising within a Meckel diverticulum.
- To highlight the diagnostic features and implications of this rare presentation.
Main Methods:
- A 44-year-old male presented with acute abdominal pain and signs of enteric obstruction.
- Diagnostic laparoscopy revealed an intact Meckel diverticulum.
- Microscopic examination, immunohistochemistry, and fluorescence in situ hybridization (FISH) were performed.
Main Results:
- Microscopy revealed extensive infiltration by monocytoid B-cells in the submucosa and serosa of the Meckel diverticulum.
- Immunohistochemistry showed positivity for CD20, BCL-2, and weak CD43, with negativity for other markers.
- FISH confirmed the API2-MALT1 fusion, characteristic of t(11;18)(q21;q21), confirming EMZL (MALT lymphoma).
Conclusions:
- Extranodal marginal zone lymphoma can occur within a Meckel diverticulum.
- This diagnosis should be considered in patients with Meckel diverticulum and unexplained gastrointestinal symptoms.
- The findings expand the known spectrum of MALT lymphoma manifestations.
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