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Plasmablastic myeloma in Taiwan frequently presents with extramedullary and extranodal mass mimicking plasmablastic lymphoma

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Abstract

Plasmablastic myeloma (PBM) is a blastic morphologic variant of plasma cell myeloma with less favorable prognosis than those with non-blastic morphology. PBM is rare, without clear-cut definition and detailed clinicopathologic features in the literature. PBM may mimic plasmablastic lymphoma (PBL) as they share nearly identical morphology and immunophenotype. Using the criteria of ≥ 30% plasmablasts in tissue sections, we retrospectively recruited PBM cases and analyzed their clinical, imaging, and pathologic findings, with emphasis on extramedullary involvement. We performed immunohistochemistry, in situ hybridization for Epstein-Barr virus (EBER), and fluorescence in situ hybridization (FISH) for lymphoma- and myeloma-associated genetic alterations. Of the 25 recruited cases, 15 (60%) had extramedullary involvement, which occurred as initial presentation in nine cases. The most common extramedullary sites were soft tissue and/or skin (10/15, 67%), followed by pleural effusion, the lungs, and lymph nodes. Immunohistochemically, tumor cells expressed MYC (74%; 17/23), CD56 (56%; 14/25), and cyclin D1 (16%; 4/25), while CD117 was all negative (n = 25). Of the 20 cases stained with p53, four (20%) cases were diffusely positive, and the remaining 16 cases showed a heterogeneous pattern. EBER was negative in all 24 cases examined. Of the 13 cases examined with FISH, the genetic aberrations identified included del(13q14)(92%; 12/13), gain of chromosome 1q (90%; 9/10), loss of chromosome 1p (60%; 6/10), IGH-FGFR3 reciprocal translocation (23%; 3/13), rearranged MYC (15%; 2/13), and rearranged CCND1 (8%; 1/13), while there were no cases with TP53 deletion (n = 10) or rearrangement of BCL2 (n = 13) or BCL6 (n = 13). The prognosis was dismal regardless of the presence or absence of extramedullary involvement. In conclusion, PBM in Taiwan frequently presented as extramedullary and extranodal lesions, particularly in soft tissue and/or skin, mimicking PBL. FISH for targeted genetic alterations such as del(13q14), gain of chromosome 1q, loss of chromosome 1p, and IGH-FGFR3 might be helpful for the differential diagnoses. Larger studies are warranted to investigate the genetic alterations between PBM and PBL.

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All data generated or analyzed during this study are included in this article and its supplementary information file.

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Funding

This study was funded by Taipei Medical University-Shuang Ho Hospital, grant no. 109TMU-SHH-15, and the Ministry of Science and Technology, Taiwan (MOST 108–2314-B-384–004).

Author information

Authors and Affiliations

  1. Department of Pathology, Shuang Ho Hospital, Taipei Medical University, New Taipei City, Taiwan

    Bo-Jung Chen

  2. Department of Pathology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan

    Bo-Jung Chen

  3. Graduate Institute of Clinical Medicine, National Taiwan University, Taipei, Taiwan

    Chang-Tsu Yuan

  4. Department of Pathology, National Taiwan University Cancer Center, Taipei, Taiwan

    Chang-Tsu Yuan

  5. Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan

    Chang-Tsu Yuan

  6. Department of Pathology and Laboratory Medicine, Taipei Veterans General Hospital, Taipei, Taiwan

    Ching-Fen Yang

  7. School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan

    Ching-Fen Yang

  8. Department of Medical Research, Chi-Mei Medical Center, Tainan, Taiwan

    Chung-Han Ho

  9. Biostatistics Center, Office of Data Science, Taipei Medical University, Taipei City, Taiwan

    Yen-Kuang Lin

  10. Graduate Institute of Data Science, College of Management, Taipei Medical University, Taipei City, Taiwan

    Yen-Kuang Lin

  11. Department of Pathology, Chi-Mei Medical Center, 901 Chung-Hwa Road, Yong-Kang District, Tainan, 71004, Taiwan

    Ying-Zhen Su & Shih-Sung Chuang

  12. Department of Anatomy and Cell Biology, School of Medicine, College of Medicine, Taipei Medical University, 250 Wu-Hsing Street, Taipei, 110, Taiwan

    Hsiu-Chu Chou

  13. Department of Information Management, Southern Taiwan University of Science and Technology, Tainan, Taiwan

    Chung-Han Ho

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  1. Bo-Jung Chen
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Contributions

HCC and SSC initiated the study; BJC, CTU, CFY, CHH, YKL, and SSC analyzed the data; YZS did the experiments; BJC and SSC wrote the manuscript; HCC and SSC revised the manuscript; all authors approved the manuscript.

Corresponding authors

Correspondence to Hsiu-Chu Chou or Shih-Sung Chuang.

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This study was approved by the Internal Review Board of Chi-Mei Medical Center, Tainan, Taiwan.

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Hsiu-Chu Chou and Shih-Sung Chuang contributed equally, and both are considered corresponding authors.

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Chen, BJ., Yuan, CT., Yang, CF. et al. Plasmablastic myeloma in Taiwan frequently presents with extramedullary and extranodal mass mimicking plasmablastic lymphoma. Virchows Arch 481, 283–293 (2022). https://doi.org/10.1007/s00428-022-03342-3

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