J Pathol Transl Med.  2024 Jul;58(4):191-197. 10.4132/jptm.2024.05.14.

Concurrent intestinal plasmablastic lymphoma and diffuse large B-cell lymphoma with a clonal relationship: a case report and literature review

• Imuta N
• Miyai K
• Tsuchiya M
• Saito M
• Sone T
• Kobayashi S
• Ogata S
• Kimura F
• Matsukuma S

• KMID: 2557759
• DOI: http://doi.org/10.4132/jptm.2024.05.14

Abstract

Herein, we report a case of plasmablastic lymphoma (PBL) and diffuse large B-cell lymphoma (DLBCL) that occurred concurrently in the large intestine. An 84-year-old female presented with a palpable rectal tumor and ileocecal tumor observed on imaging analyses. Endoscopic biopsy of both lesions revealed lymphomatous round cells. Hartmann’s operation and ileocecal resection were performed for regional control. The ileocecal lesion consisted of a proliferation of CD20/CD79a-positive lymphoid cells, indicative of DLBCL. In contrast, the rectal tumor showed proliferation of atypical cells with pleomorphic nuclei and abundant amphophilic cytoplasm, with immunohistochemical findings of CD38/CD79a/MUM1/MYC (+) and CD20/CD3/CD138/PAX5 (–). Tumor cells were positive for Epstein-Barr virus– encoded RNA based on in situ hybridization and MYC rearrangement in fluorescence in situ hybridization analysis. These findings indicated the rectal tumor was most likely a PBL. Sequencing analysis for immunoglobulin heavy variable genes indicated a common B-cell origin of the two sets of lymphoma cells. This case report and literature review provide new insights into PBL tumorigenesis.

Keyword

Plasmablastic lymphoma; Diffuse large B-cell lymphoma; Concurrent lymphoma; Sequencing analysis; Clonal relationship

Figure

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  Fig. 1. Gross, microscopic, and immunohistochemical findings of the ileocecal tumor. (A) An ileocecal tumor, 40-mm in size, involving the cecum and appendix vermiformis (arrow). (B) The cut surface of the resected specimen showing a white solid tumor with luminal bleeding. (C) The tumor composed of a diffuse proliferation of medium- to large-sized lymphoid cells with prominent nucleoli. (D–F) The lymphoid cells were diffusely immunoreactive for CD20 (D) and CD79a (E) and negative for CD3 (F).

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  Fig. 2. Gross, microscopic, and immunobiological findings of the rectal tumor. (A) A 55-mm-sized ulcerating localized rectal tumor in the surgically resected specimen (B) The cut surface of the rectal tumor involving the whole rectal wall. (C) The tumor showing a dense cellular infiltration with geographic coagulative necrosis. (D) The tumor consisting of highly atypical lymphoid cells with pleomorphic nuclei and abundant amphophilic cytoplasm. Mitotic figures were frequently observed. (E–G) Immunohistochemically, tumor cells were (E, left) CD20 (−), (E, right) CD79a (+), (F, left) CD138 (−), (F, right) CD38 (+), (G, left) MUM1 (+), and (G, right) MYC (+, > 40%). (H) Tumor cells were positive for in situ hybridization of Epstein-Barr virus-encoded small RNA.

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  Fig. 3. Fluorescence in situ hybridization and sequencing analyses. (A, B) Split signals indicating MYC rearrangement were detected in tumor cells of the rectal tumor (A) but not in those of the cecal tumor (B). (C) Sequences of the complementarity-determining region 3 (CDR3) region in the immunoglobulin heavy variable genes of the ileocecal tumor and rectal tumor. The sequence of the CDR3 region in the two tumors matched. Genomic alterations from reference (NC_000014.9) are indicated as red letters.

Reference

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