Clinicopathologic and Molecular Characterization of Epstein-Barr Virus-positive Plasmacytoma

Epstein-Barr virus (EBV)-positive plasmacytoma is a rare plasma cell neoplasm. It remains unclear whether EBV-positive plasmacytoma represents a distinct entity or a variant of plasmacytoma. It shares morphologic features with plasmablastic lymphoma (PBL) and may cause diagnostic uncertainty. To bet...

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Published in:The American journal of surgical pathology Vol. 46; no. 10; p. 1364
Main Authors: Zhou, Ting, Cheng, Jinjun, Karrs, Jeremiah, Davies-Hill, Theresa, Pack, Svetlana D, Xi, Liqiang, Tyagi, Manoj, Kim, Jung, Jaffe, Elaine S, Raffeld, Mark, Pittaluga, Stefania
Format: Journal Article
Language:English
Published: United States 01.10.2022
Subjects:
Epstein-Barr Virus Infections - complications
Epstein-Barr Virus Infections - diagnosis
Herpesvirus 4, Human - genetics
Humans
In Situ Hybridization, Fluorescence
Ki-67 Antigen
Plasmacytoma - complications
Plasmacytoma - diagnosis
Plasmacytoma - genetics
ISSN:1532-0979, 1532-0979
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Summary:Epstein-Barr virus (EBV)-positive plasmacytoma is a rare plasma cell neoplasm. It remains unclear whether EBV-positive plasmacytoma represents a distinct entity or a variant of plasmacytoma. It shares morphologic features with plasmablastic lymphoma (PBL) and may cause diagnostic uncertainty. To better understand EBV-positive plasmacytoma and explore diagnostic criteria, this study describes 19 cases of EBV-positive plasmacytoma, compared with 27 cases of EBV-negative plasmacytoma and 48 cases of EBV-positive PBL. We reviewed the clinicopathologic findings and performed immunohistochemistry, in situ hybridization for EBV, fluorescence in situ hybridization for MYC , and next-generation sequencing. We found that 63.2% of patients with EBV-positive plasmacytoma were immunocompromised. Anaplastic features were observed in 7/19 cases. MYC rearrangement was found in 25.0% of them, and extra copies of MYC in 81.3%. EBV-positive and EBV-negative plasmacytomas possessed similar clinicopathologic features, except more frequent cytologic atypia, bone involvement and MYC aberrations in the former group. The survival rate of patients with EBV-positive plasmacytoma was comparable to that of patients with EBV-negative plasmacytoma. In comparison to PBL, EBV-positive plasmacytoma is less commonly associated with a "starry-sky" appearance, necrosis, absence of light chain expression, and a high Ki67 index (>75%). The most recurrently mutated genes/signaling pathways in EBV-positive plasmacytoma are epigenetic regulators, MAPK pathway, and DNA damage response, while the most frequently reported mutations in PBL are not observed. Collectively, EBV-positive plasmacytoma should be regarded as a biological variant of plasmacytoma. Thorough morphologic examination remains the cornerstone for distinguishing EBV-positive plasmacytoma and PBL, and molecular studies can be a valuable complementary tool.
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ISSN:1532-0979
1532-0979
DOI:10.1097/PAS.0000000000001923