scholarly journals Epstein-Barr virus-positive mucocutaneous ulcer colliding with extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (malt lymphoma) that developed in the palatine tonsils of an immunocompetent patient after gastric lymphoma relapse

2018 ◽  
Vol 53 (4) ◽  
pp. 329 ◽  
Author(s):  
Yong-Moon Lee ◽  
Jin-Man Kim
Keyword(s):  
Malt Lymphoma ◽  
Lymphoid Tissue ◽  
Epstein Barr Virus ◽  
Marginal Zone ◽  
Gastric Lymphoma ◽  
Immunocompetent Patient ◽  
Marginal Zone Lymphoma ◽  
Palatine Tonsils ◽  
Barr Virus ◽  
Epstein Barr

Gastric marginal zone lymphoma of mucosa-associated tissue and Epstein-Barr Virus associated lymphoepithelioma gastric carcinoma: Two primaries with an unusual metachronous presentation

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S68-S69
Author(s):  
J Gallardo ◽  
J Mahadik ◽  
C J Finch ◽  
R Szigeti ◽  
J Shilpa
Keyword(s):  
T Lymphocytes ◽  
Epithelial Cells ◽  
Gastric Carcinoma ◽  
Malt Lymphoma ◽  
Epstein Barr Virus ◽  
Marginal Zone ◽  
Marginal Zone Lymphoma ◽  
Barr Virus ◽  
Epstein Barr ◽  
Lymphoepithelial Lesions

Abstract Introduction/Objective Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) and Epstein-Barr virus (EBV) lymphoepithelioma gastric carcinoma, are two distinct gastric malignancies, with well-known clinicopathological characteristics. Synchronous or metachronous presentation of both entities in the same patient is extremely rare. Methods/Case Report We report a case of a 78-year-old woman, who presented with a 3 cm gastric body mass. Histologically, the biopsy showed H. pylori gastritis, with diffuse monotonous atypical lymphocytic cells and prominent lymphoepithelial lesions. CD20 immunostain was diffusely positive in these cells, supporting the diagnosis of MALT lymphoma. The patient was treated for H. pylorieradication, followed by Rituxan for resistant MALT lymphoma found in a surveillance biopsy. After being free of disease for one year, the patient developed a 2.0 cm, ulcerated lesion adjacent to the previous site. Biopsy of this lesion showed gastric mucosa with diffuse lymphoepithelial lesions, and atypical epithelial cells highlighted by pan-cytokeratin. The associated dense inflammatory infiltrate was comprised predominantly of CD3 T-lymphocytes. EBV was detected by in-situ hybridization (ISH) for EBV encoded RNA, and was positive in the epithelial cells, but negative in lymphocytes. These findings are consistent with EBV associated lymphoepithelioma carcinoma. Results (if a Case Study enter NA) NA Conclusion This case is presented due to its rarity, with only two cases reported previously, which invokes further research into the interaction between both infectious agents. Secondly, lymphoepithelial lesions are a common finding in both diseases, and in small biopsies, these entities can mask or mimic each other. EBV ISH and background B and T lymphocytes may be a clue and help in the diagnosis.

scholarly journals Case Report: EBV-Positive Extra-Nodal Marginal Zone Lymphoma Associated With XMEN Disease Caused by a Novel Hemizygous Mutation in MAGT1

2021 ◽  
Vol 11 ◽  
Author(s):  
Xin Huang ◽  
Dan Liu ◽  
Zifen Gao ◽  
Cuiling Liu
Keyword(s):  
Epstein Barr Virus ◽  
Marginal Zone ◽  
Pediatric Population ◽  
Genetic Disorder ◽  
Marginal Zone Lymphoma ◽  
Epstein Barr Virus Infection ◽  
Loss Of Function ◽  
Barr Virus ◽  
Epstein Barr ◽  
Nodal Marginal Zone Lymphoma

BackgroundX-linked immunodeficiency with magnesium defect and Epstein-Barr virus infection and neoplasia (XMEN) disease is an X-linked genetic disorder of immune system caused by loss-of-function mutation in gene encoding Magnesium transporter 1 (MAGT1). Individuals with XMEN disease are prone to developing Epstein Barr Virus (EBV)-associated lymphomas. Herein, we report the first known case of an EBV+ EMZL associated with XMEN disease.Case presentationThe patient was an 8-year-old Chinese boy who suffered from recurrent infections from birth. Six months before, the patient presented with a painless mass on his upper lip and excisional biopsy revealed an EBV-positive extra-nodal marginal zone lymphoma (EBV+ EMZL). Furthermore, molecular investigations with next-generation sequencing identified a novel germline mutation in MAGT1 (c.828_829insAT) in the patient. The c.828_829insAT variant was predicted to cause premature truncation of MAGT1 (p.A277M.fs*11) and consequently was defined as likely pathogenic. The mutation was inherited from his asymptomatic heterozygous carrier mother. Hence the patient was diagnosed with an XMEN disease both clinically and genetically.ConclusionOur results expand the genetic spectrum of XMEN disease and also the clinical spectrum of EBV+ EMZL. We highlight the importance of the genetic etiology underlying EBV+ lymphoma in the pediatric population.

Epstein-Barr Virus–Associated Nodal Marginal Zone Lymphoma: Part of the Spectrum of Posttransplant Lymphoproliferative Disorder?

2018 ◽  
Vol 27 (1) ◽  
pp. 94-97
Author(s):  
Chi Yuen Cheung ◽  
Wing Hung Lau ◽  
Wah Cheuk
Keyword(s):  
Lymphoproliferative Disorder ◽  
Epstein Barr Virus ◽  
Marginal Zone ◽  
Marginal Zone Lymphoma ◽  
Lymphoid Cells ◽  
Posttransplant Lymphoproliferative Disorder ◽  
Barr Virus ◽  
The Status ◽  
Epstein Barr ◽  
Nodal Marginal Zone Lymphoma

A 56-year-old man, who received deceased kidney transplant 20 years ago, presented with an enlarged submandibular lymph node. Histologic examination revealed nodal marginal zone lymphoma in which the neoplastic lymphoid cells showed diffuse positivity for Epstein-Barr virus early RNA by in situ hybridization. Systemic lymphoma workup showed stage I disease. The tumor was managed as a posttransplant lymphoproliferative disorder and the immunosuppression was modified. There was no evidence of lymphoma at follow-up 6 years after excision alone. This case supports the inclusion of Epstein-Barr virus–positive nodal marginal zone lymphoma as a form of monomorphic B-cell lymphoproliferative disorder, in line with the status of its extranodal mucosa-associated lymphoid tissue lymphoma counterpart.

Genomic landscape of Epstein–Barr virus-positive extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue

2021 ◽  
Author(s):  
Bryan Rea ◽  
Yen-Chun Liu ◽  
Alanna Maguire ◽  
Lorinda A. Soma ◽  
Chris M. Bacon ◽  
...  
Keyword(s):  
Lymphoid Tissue ◽  
Epstein Barr Virus ◽  
Marginal Zone ◽  
Barr Virus ◽  
Genomic Landscape ◽  
Epstein Barr

Epstein-Barr Virus–Associated High-Grade B-Cell Lymphoma of Mucosal-Associated Lymphoid Tissue in a 9-Year-Old Boy

2000 ◽  
Vol 124 (10) ◽  
pp. 1520-1524 ◽  
Author(s):  
Jianguo Tao ◽  
Leonard Kahn
Keyword(s):  
B Cell ◽  
Malt Lymphoma ◽  
Lymphoid Tissue ◽  
Epstein Barr Virus ◽  
B Cell Lymphoma ◽  
Low Grade ◽  
Renal Tubules ◽  
High Grade ◽  
Barr Virus ◽  
Epstein Barr

Abstract We report an unusual case of Epstein-Barr virus (EBV)-associated mucosal-associated lymphoid tissue (MALT) lymphoma involving the lungs, kidneys, and axillary lymph nodes in a child with congenital hypoadrenalism and panhypopituitarism. The patient presented with an aggressive clinical course and histologic evolution. Initial biopsies (1994) of the lung and kidney revealed histologic features of low-grade B-cell MALT lymphoma with lymphoepithelial lesions within the renal tubules and bronchial epithelium. Subsequent biopsies (1996, 1997, and 1999) revealed progressively greater cytologic atypia, polymorphism, and necrosis; an increased mitotic rate; and a preponderance of large cells, indicative of progression from a low-grade to a high-grade MALT lymphoma. Immunophenotyping of the lung and lymph node lesions revealed identical surface marker profiles: cells were CD19+, CD20+, immunoglobulin (Ig) G+, κ+, λ−, CD5−, CD10−, CD23−, and IgM−, and also negative for T-cell markers. Genotypic analysis demonstrated the presence of immunoglobulin heavy chain rearrangement and monoclonality of EBV in the lung lesion by Southern blot hybridization and polymerase chain reaction (PCR). The clinicopathologic features suggest that these lesions might represent an immunosupression-related continuum of low-grade to high-grade MALT lymphomas. Infection with EBV may have contributed to this tumor's aggressive clinical and histologic evolution.

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