Epstein–Barr virus-positive ileal extraosseous plasmacytoma containing plasmablastic lymphoma components with CD20-positive lymph node involvement

Abstract

We report a case of Epstein–Barr virus (EBV)-positive ileal extraosseous plasmacytoma containing plasmablastic lymphoma components with CD20-positive lymph node involvement. A 34-year-old healthy Japanese male developed intussusception due to an ileal plasmacytoma. The lesion was positive for EBV-encoded small nuclear RNA in in situ hybridization, with the surrounding lymph nodes showing the expression of CD20. Tumor cells in the ileal and lymph node lesions contained high-grade malignant features compatible with plasmablastic lymphoma. Because his abdominal lymph nodes recurred 6 months after resection, he received six cycles of R-CHOP chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone), and had a complete remission. Although his case was complicated by acute promyelocytic leukemia, he has so far survived, recurrence-free, for more than 7.5 years after chemotherapy for extraosseous plasmacytoma.

Epstein–Barr virus‐positive plasmacytoma in immunocompetent patients

“…The head and neck and the gastrointestinal tract were the most common sites. Little information is available in the literature regarding therapy, follow‐up, or survival, with the exceptions of a case reported by Saito et al . and the cases that we report here.…”

“…EBV‐positive plasmacytomas in immunocompetent hosts have been described in the literature, and are summarized in Table . Including the four cases that we report here, a total of 11 cases of EBV‐positive plasmacytoma have been reported.…”

“…In addition, none of the four patients included in this study had signs or symptoms of chronic active EBV infection, such as fever, generalized lymphadenopathy, hepatosplenomegaly, cytopenias, or liver function abnormalities. 35 EBV-positive plasmacytomas in immunocompetent hosts have been described in the literature, 25,[36][37][38] and are summarized in Table 4. Including the four cases that we report here, a total of 11 cases of EBVpositive plasmacytoma have been reported.…”

“…The head and neck and the gastrointestinal tract were the most common sites. Little information is available in the literature regarding therapy, follow-up, or survival, with the exceptions of a case reported by Saito et al 38 Although EBV infection is a confounding variable, the distinction between plasmacytoma and PBL can be made on the basis of histopathological examination. In general, plasmacytoma is composed of mature plasma cells with minimal atypia and low mitotic and proliferation rates, although a subset of cases can show more atypia and mitotic figures.…”

Plasmablastic transformation of a pre-existing plasmacytoma: a possible role for reactivation of Epstein Barr virus infection

“…2 Although it has been suggested that PBL may occur as a high-grade transformation from a pre-existing plasmacytoma following EBV infection, this concept has not been well established and definitive proof is lacking. 3,4 In December 2011, a 74-year old man presented for a right maxillary sinus solitary mass, infiltrating the oral cavity. Histological examination of the bioptic specimen showed a uniform proliferation of plasma cells with abundant cytoplasm, round eccentrically located nuclei and clock-face chromatin without nucleoli ( Figure 1A).…”