Epstein-Barr Virus–associated Extranodal Marginal Zone Lymphoma of Mucosa-associated Lymphoid Tissue (MALT Lymphoma) Arising in the Parotid Gland of a Child With Ataxia Telangiectasia

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.

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Primary IgG4‐producing extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue (MALT lymphoma) in the cavernous sinus: A mimicker of IgG4‐related disease/hypertrophic pachymeningitis

Pathology International ◽

10.1111/pin.13070 ◽

2021 ◽

Author(s):

Chao‐Hung Yeh ◽

Yu‐Kun Tsui ◽

Hongxiang Liu ◽

Shih‐Sung Chuang

Keyword(s):

Cavernous Sinus ◽

Malt Lymphoma ◽

Lymphoid Tissue ◽

Marginal Zone ◽

Marginal Zone Lymphoma ◽

Hypertrophic Pachymeningitis ◽

Extranodal Marginal Zone Lymphoma ◽

Related Disease ◽

Igg4 Related Disease

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Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)

Hematologic Malignancies - Indolent Lymphomas ◽

10.1007/978-3-030-55989-2_7 ◽

2021 ◽

pp. 93-115

Author(s):

Emanuele Zucca ◽

Markus Raderer

Keyword(s):

Malt Lymphoma ◽

Lymphoid Tissue ◽

Marginal Zone ◽

Marginal Zone Lymphoma ◽

Extranodal Marginal Zone Lymphoma

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Helicobacter pyloriinfection in gastric extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma: a re-evaluation

Gut ◽

10.1136/gutjnl-2014-307389 ◽

2014 ◽

Vol 63 (9) ◽

pp. 1526-1527 ◽

Cited By ~ 15

Author(s):

Larissa Sena Teixeira Mendes ◽

Ayoma D Attygalle ◽

Andrew C Wotherspoon

Keyword(s):

Malt Lymphoma ◽

Lymphoid Tissue ◽

Marginal Zone ◽

Marginal Zone Lymphoma ◽

Extranodal Marginal Zone Lymphoma

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

Frontiers in Oncology ◽

10.3389/fonc.2021.653266 ◽

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.

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B-Cell Monoclonality, Epstein Barr Virus, and t(14; 18) in Myoepithelial Sialadenitis and Low-Grade B-Cell MALT Lymphoma of the Parotid Gland

The American Journal of Surgical Pathology ◽

10.1097/00000478-199505000-00004 ◽

1995 ◽

Vol 19 (5) ◽

pp. 531-536 ◽

Cited By ~ 76

Author(s):

Timothy C. Diss ◽

Andrew C. Wotherspoon ◽

Paul Speight ◽

Langxing Pan ◽

Peter G. Isaacson

Keyword(s):

Parotid Gland ◽

B Cell ◽

Malt Lymphoma ◽

Epstein Barr Virus ◽

Low Grade ◽

Barr Virus ◽

Epstein Barr

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Primary Hepatic Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue in a Patient with Chronic Hepatitis B Virus Infection: Case Report and Summary of the Literature

Medicina ◽

10.3390/medicina57030280 ◽

2021 ◽

Vol 57 (3) ◽

pp. 280

Author(s):

Yuki Yamashita ◽

Satoru Joshita ◽

Hiroyuki Kobayashi ◽

Shun-ichi Wakabayashi ◽

Ayumi Sugiura ◽

...

Keyword(s):

Hepatitis B ◽

Malt Lymphoma ◽

Lymphoid Tissue ◽

Marginal Zone ◽

Disease Diagnosis ◽

Marginal Zone Lymphoma ◽

Focal Liver Lesions ◽

Liver Lesions ◽

Extranodal Marginal Zone Lymphoma ◽

Chronic Hepatitis B Virus

Background: The incidence of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is low, at 7–8% of all non-Hodgkin lymphoma cases. The most common site of MALT lymphoma occurrence is the stomach. Primary hepatic extranodal marginal zone lymphoma of MALT is classified as a type of non-gastric MALT lymphoma and is considered extremely rare, with no consensus on imaging study findings or treatment due to a limited number of reports. We herein describe a rare case of primary hepatic extranodal marginal zone lymphoma of MALT with underlying hepatitis B infection (HBV) and present useful diagnostic findings of various imaging modalities, including contrast-enhanced ultrasonography (CEUS) with Sonazoid. Case presentation: A 66-year-old woman was diagnosed as being a non-active carrier of HBV at 51 years of age at the time of total hysterectomy and bilateral adnexectomy for uterine cervical cancer. She was admitted to our hospital following the incidental detection of two focal liver lesions on computed tomography. The lesions were considered malignant based on clinical and other radiologic imaging findings. Her CEUS results of hypo-enhancement in the portal and late phases were consistent with those of previously reported cases of hepatic extranodal marginal zone lymphoma of MALT, and histological liver biopsy findings were compatible with the diagnosis. Conclusions: Primary hepatic extranodal marginal zone lymphoma of MALT is a rare condition that can appear in HBV carriers. Characteristic CEUS findings may help in disease diagnosis. Clinicians should bear primary hepatic extranodal marginal zone lymphoma of MALT in mind when encountering patients with focal liver lesions which exhibit image findings different from those of typical hepatocellular carcinoma.

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