EBV positive mucocutaneous ulcer with plasmacytic/plasmablastic differentiation and MYC rearrangement: a diagnostic challenge and a mimic of plasmablastic lymphoma

Chen, Bo-Jung; Chiang, Wei‐Fan; Chen, Tsang-Shan; Kao, Hsu-Ju; Chuang, Shih‐Sung

doi:10.1016/j.pathol.2019.07.003

Cited by 8 publications

(12 citation statements)

References 6 publications

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“…Several morphologic patterns have been described for EBV mucocutaneous ulcer to date, and include: polymorphous, large cell-rich, classic Hodgkin lymphoma-like, mucosa-associated lymphoid tissue (MALT) lymphoma-like, EBV mucocutaneous ulcer with small lymphocytic infiltration and EBV mucocutaneous ulcer with plasmacytic/plasmablastic immunophenotype 5,6,8,14. In this case report, the pathology fits with the classic Hodgkin lymphoma-like pattern.…”

Section: Discussionsupporting

confidence: 51%

“…Commonly involved sites included the oropharyngeal tract, skin and gastrointestinal tract. Since then, emerging publications have expanded our understanding of the clinical, morphologic and molecular spectrum of this entity (2)(3)(4)(5)(6). It now includes human immunodeficiency virus (HIV) infection as etiology, multiple lesions within an anatomic region, and a widened spectrum of morphologic patterns.…”

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confidence: 99%

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Epstein-Barr Virus-positive Mucocutanous Ulcer of the Uterine Cervix: Report of a Rare and Evolving Entity

Razack

Cariem

Irusen

et al. 2022

International Journal of Gynecological Pathology

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Epstein-Barr virus (EBV)-positive mucocutaneous ulcer (EBVMCU) is a newly described lymphoproliferative lesion characterized by the proliferation of EBV-positive atypical B cells in a variable inflammatory background associated with mucosal or cutaneous ulcers. Commonly involved sites include the oropharyngeal tract, skin, and gastrointestinal tract, with emerging reports describing EBVMCU of genitalia. To date, no lesions have been described of the uterine cervix. Herein, we report the first cervical EBVMCU incidentally discovered at large loop excision of the transformation zone performed for treating human papillomavirus induced high-grade squamous intraepithelial lesion. The patient was a 35-yr-old, human immunodeficiency virus-positive woman with a history of pulmonary tuberculosis. Histologic and immunohistochemical findings showed classic Hodgkin lymphoma-like features. Systemic lymphoproliferative disease was excluded by appropriate clinical and imaging modalities. This case report highlights the prototypical histologic features of cervical EBVMCU. We emphasize the importance of clinicopathologic correlation to avoid overtreatment of a lesion that can otherwise meet histologic criteria for a lymphoma. Clinicians and pathologists should familiarize themselves with this entity, as this indolent, pseudomaligant lesion typically occurs in immunocompromised patients and spontaneously regresses when the cause for the immunosuppression is addressed.

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Section: Discussionsupporting

confidence: 51%

mentioning

confidence: 99%

Epstein-Barr Virus-positive Mucocutanous Ulcer of the Uterine Cervix: Report of a Rare and Evolving Entity

Razack

Cariem

Irusen

et al. 2022

International Journal of Gynecological Pathology

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“…In our previous study, 9% of the EBVMCU cases belonged to this subtype [53]. Another study reported an EBVMCU case that resembled plasmablastic lymphoma (PBL) [56]. Furthermore, some studies have reported good prognoses among patients diagnosed with HIV-negative PBL [70][71][72][73][74].…”

Section: Mucosa-associated Lymphoid Tissue (Malt) Lymphoma-likementioning

confidence: 93%

Epstein–Barr Virus-Positive Mucocutaneous Ulcer: A Unique and Curious Disease Entity

Ikeda

Gion

Nishimura

et al. 2021

IJMS

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Epstein–Barr virus (EBV)-positive mucocutaneous ulcer (EBVMCU) was first described as a lymphoproliferative disorder in 2010. EBVMCU is a unifocal mucosal or cutaneous ulcer that often occurs after local trauma in patients with immunosuppression; the patients generally have a good prognosis. It is histologically characterized by proliferating EBV-positive atypical B cells accompanied by ulcers. On the basis of conventional pathologic criteria, EBVMCU may be misdiagnosed as EBV-positive diffuse large B-cell lymphoma or other lymphomas. However, its prognosis differs from that of EBV-associated lymphomas, in that patients with EBVMCU frequently show spontaneous regression or complete remission without chemotherapy. Therefore, EBVMCU is now recognized as a low-grade malignancy or a pseudo-malignant lesion. Avoiding unnecessary chemotherapy by distinguishing EBVMCU from other EBV-associated lymphomas will reduce the burden and unnecessary harm on patients. On the basis of these facts, EBVMCU was first described as a new clinicopathological entity by the World Health Organization in 2017. In this review, we discuss the clinicopathological characteristics of previously reported EBVMCU cases, while focusing on up-to-date clinical, pathological, and genetic aspects.

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“…Based on its wide histological variations; however, differential diagnosis of EBV-MCU includes such malignant cutaneous lymphomas as DLBCL, classic Hodgkin’s lymphoma and mucosa-associated lymphoid tissue lymphoma 18 19. As was so in the current case, EBV-MCU with plasmacytic/plasmablastic differentiation microscopically mimics plasmablastic lymphoma 19 21. It has been emphasised that the histopathological distinction between EBV-MCU and aggressive EBV-positive cutaneous lymphoma may be impossible without careful clinical evaluations 22 23…”

Section: Discussionmentioning

confidence: 67%

“…There are significant histological variations in EBV-MCU, including prototypic polymorphous type, large cell-rich type resembling DLBCL, classic Hodgkin’s lymphoma-like type and mucosa-associated lymphoid tissue lymphoma-like type 18 19. EBV-MCU with plasmacytic/plasmablastic differentiation19 21 mimicking plasmablastic lymphoma, as well as EBV-MCU with small lymphocytic infiltration mimicking non-specific ulceration,22 have also been described. The distinction between aggressive EBV-positive cutaneous lymphoma and EBV-MUC is often difficult by histopathologic evaluation alone 22 23.…”

Section: Introductionmentioning

confidence: 99%

Epstein-Barr virus-positive mucocutaneous ulcer, plasmablastic type, associated with nodal CD4+ angioimmunoblastic T-cell lymphoma and generalised pruritus: a self-limiting lymphoproliferative disorder resembling cutaneous plasmablastic lymphoma

et al. 2022

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A woman in her 80s reported of generalised pruritus, which was treated with phototherapy and steroid administration. Two months after onset, lymph node biopsy revealed CD4+ angioimmunoblastic T-cell lymphoma with systemic superficial nodal involvement. Intractable prurigo was judged as T-cell lymphoma related. After effective chemotherapy (7 months later), skin nodules appeared multifocally, including on the lip, thumb and lower leg. The biopsied infiltrative lesion on the right lower leg microscopically revealed subcutaneous growth of atypical plasmablasts with nearly 100% Ki-67 labelling and Epstein-Barr virus (EBV)-encoded small nuclear RNA positivity. Plasmablastic lymphoma (CD45/CD19/CD38/CD138/MUM1+, CD20/CD79a/PAX5-) was suspected. Immunoglobulin light-chain restriction and nuclear expression of c-myc protein were undetectable, and the ulcers were spontaneously epithelialised by the cessation of steroid administration. After 10 months, non-progressive prurigos persisted on the extremities, but without regrowth of nodal T-cell lymphoma and cutaneous lymphoproliferative lesion. Reactive nature of the EBV-induced mucocutaneous plasmablastic growth (EBV-positive mucocutaneous ulcer, plasmablastic type) is discussed.

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EBV positive mucocutaneous ulcer with plasmacytic/plasmablastic differentiation and MYC rearrangement: a diagnostic challenge and a mimic of plasmablastic lymphoma

Cited by 8 publications

References 6 publications

Epstein-Barr Virus-positive Mucocutanous Ulcer of the Uterine Cervix: Report of a Rare and Evolving Entity

Epstein-Barr Virus-positive Mucocutanous Ulcer of the Uterine Cervix: Report of a Rare and Evolving Entity

Epstein–Barr Virus-Positive Mucocutaneous Ulcer: A Unique and Curious Disease Entity

Epstein-Barr virus-positive mucocutaneous ulcer, plasmablastic type, associated with nodal CD4+ angioimmunoblastic T-cell lymphoma and generalised pruritus: a self-limiting lymphoproliferative disorder resembling cutaneous plasmablastic lymphoma

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