Extranodal Marginal Zone Lymphoma Presenting within the Meckel Diverticulum as Diverticulitis: A Case Report

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.

[1]  Jun Gi Kim,et al.  Adenocarcinoma arising from Meckel's diverticulum in the ileum with malrotation of the midgut , 2013, Journal of the Korean Surgical Society.

[2]  J. V. van Krieken,et al.  T(14;18)(q32;q21) involving MALT1 and IGH genes occurs in extranodal diffuse large B-cell lymphomas of the breast and testis , 2013, Modern Pathology.

[3]  J. Coebergh,et al.  Trends in incidence, therapy and outcome of localized nodal and extranodal marginal zone lymphomas: declining incidence and inferior outcome for gastrointestinal sites , 2013, Leukemia & lymphoma.

[4]  Y. Ishigatsubo,et al.  Clinical features of extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue , 2012, Hematological oncology.

[5]  V. Mehta,et al.  Primary signet-ring cell carcinoma of Meckel's diverticulum: an uncommon cause of abdominal pain. , 2012, Pathology.

[6]  A. Wotherspoon,et al.  Diagnosis and management of rare gastrointestinal lymphomas , 2012, Leukemia & lymphoma.

[7]  K. Mitura,et al.  Perforated gist of Meckel's diverticulum. , 2012, Polski przeglad chirurgiczny.

[8]  J. V. van Krieken,et al.  Molecular pathogenesis and histologic and clinical features of extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue type , 2012, Leukemia & lymphoma.

[9]  E. M. Tazi,et al.  Lymphomes malins non hodgkiniens de l’intestin grêle de type « immunoproliferative small intestinal disease » , 2011 .

[10]  M. Loukas,et al.  Meckel's diverticulum: A review , 2011, Clinical anatomy.

[11]  Guangyao Wu,et al.  Primary gastrointestinal lymphoma. , 2011, World journal of gastroenterology.

[12]  M. Abid,et al.  [Complications of Meckel's diverticulum. Report of 42 cases]. , 2009, La Tunisie medicale.

[13]  M. Gatt,et al.  Mucosa associated lymphoid tissue lymphoma presenting within a solitary anti-mesenteric dilated segment of ileum: a case report , 2009, Journal of medical case reports.

[14]  N. Shinton.  WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues , 2007 .

[15]  A. Chott,et al.  T(3;14)(p14.1;q32) involving IGH and FOXP1 is a novel recurrent chromosomal aberration in MALT lymphoma , 2005, Leukemia.

[16]  P. Isaacson.  Update on MALT lymphomas. , 2005, Best practice & research. Clinical haematology.

[17]  H. Stein,et al.  Atypical marginal zone hyperplasia of mucosa-associated lymphoid tissue: a reactive condition of childhood showing immunoglobulin lambda light-chain restriction. , 2004, Blood.

[18]  R. Siebert,et al.  Variable frequencies of MALT lymphoma-associated genetic aberrations in MALT lymphomas of different sites , 2004, Leukemia.

[19]  V. Swaroop.  Immunoproliferative small intestinal disease , 2000, American Journal of Gastroenterology.

[20]  G. Salles,et al.  Mucosa-associated lymphoid tissue lymphoma is a disseminated disease in one third of 158 patients analyzed. , 2000, Blood.

[21]  Arnold Jf,et al.  Meckel's diverticulum: a ten-year experience. , 1997 .

[22]  A. Al-Dabbagh,et al.  Primary lymphoma of meckle diverticulum: A case report , 1985 .

[23]  A. Khojasteh,et al.  Current concepts immunoproliferative small intestinal disease. A "Third-World lesion". , 1983 .

[24]  I. Dawson,et al.  Primary malignant lymphoid tumours of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis , 1961, The British journal of surgery.

[25]  Chao-Chiang Tu,et al.  A clinically-occult gastrointestinal stromal tumor in a Meckel's diverticulum presenting as hollow organ perforation. , 2011, Chang Gung medical journal.

[26]  H. Errihani,et al.  [Intestinal non-Hodgkin lymphoma: "immunoproliferative small intestinal disease"]. , 2011, Presse medicale.

[27]  M. Abid,et al.  COMPLICATIONS DU DIVERTICULE DE MECKEL: À PROPOS DE 42 CAS , 2009 .

[28]  N. Cross,et al.  WHO classification of tumours of haematopoietic and lymphoid tissues Chronic myeloproliferative diseases Myelodysplastic / myeloproliferative diseases Chronic myelogenous leukaemia ( CML ) Chronic myelomonocytic leukaemia ( CMML ) Chronic neutrophilic leukaemia Atypical chronic myeloid leukaemia Chr , 2007 .

[29]  A. Avilés,et al.  Surgery and chemotherapy versus chemotherapy as treatment of high-grade MALT gastric lymphoma , 2006, Medical oncology.

[30]  M. Parrens,et al.  [Extranodal lymphoma]. , 1998, Annales de pathologie.

[31]  J. Pellicane,et al.  Meckel's diverticulum: a ten-year experience. , 1997, The American surgeon.

[32]  S. Leeper,et al.  Massive lower gastrointestinal hemorrhage in an AIDS patient: first case report of ulcerated lymphoma in a Meckel's diverticulum. , 1994, The American journal of gastroenterology.

[33]  B. Silvestri,et al.  [Primary gastrointestinal lymphoma]. , 1991, G.E.N.

[34]  W. Leisering.  [Complications of the Meckel's diverticulum]. , 1975, Zentralblatt für Chirurgie.