Department of Pathology, University of Yamanashi, Chuo, Yamanashi, Japan.
Department of Hematology & Oncology, University of Yamanashi, Chuo, Yamanashi, Japan.
J Clin Exp Hematop. 2024;64(3):268-272. doi: 10.3960/jslrt.24038.
Plasmacytoma is defined as a plasma cell neoplasm forming a solitary osseous or extramedullary tumor without evidence of myeloma or organ damage related to a plasma cell neoplasm. Epstein-Barr virus (EBV) is associated with various B-cell neoplasms, particularly in patients with immune dysregulation; however, plasmacytoma is typically negative for EBV. Here, a case of EBV-positive sternal plasmacytoma in an immunocompetent female is presented. A 76-year-old female with no immunodeficiency presented with a tumor on the anterior thoracic wall. Imaging analysis revealed a 6.3 cm-sized tumor at the manubrium, and a needle biopsy was performed. The tumor in the bone was composed of a diffuse proliferation of plasmacytes with eccentric nuclei and a perinuclear halo. By immunohistochemistry and in situ hybridization, tumor cells were CD20-, CD3-, CD138+, κ+, λ-, EBER+, and the Ki67-labeling index was approximately 20%. Subsequent studies identified IgG κ monoclonal protein in serum but no evidence of plasma cell neoplasm-related organ damage, such as hypercalcemia, anemia, or renal dysfunction. No plasma cell neoplasm was detected in the bone marrow in the morphological and flowcytometric studies. Accordingly, the diagnosis was EBV-positive plasmacytoma. The patient was treated with local radiation therapy and achieved complete remission. EBV-positive plasmacytoma is rare in immunocompetent patients and should be carefully distinguished from plasmablastic lymphoma, another EBV-positive neoplasm with a plasma cell phenotype and an aggressive clinical course. This case also raises an important question: "when to perform EBER in situ hybridization in diagnosing plasma cell neoplasm?", which prompts further large case-series studies.
浆细胞瘤定义为形成孤立性骨或骨髓外肿瘤的浆细胞肿瘤,无骨髓瘤或与浆细胞肿瘤相关的器官损害证据。EB 病毒(EBV)与各种 B 细胞肿瘤有关,特别是在免疫失调患者中;然而,浆细胞瘤通常为 EBV 阴性。在此,报告一例免疫功能正常女性的 EBV 阳性胸骨浆细胞瘤。一位 76 岁女性,无免疫缺陷,出现前胸壁肿瘤。影像学分析显示胸骨柄处有一个 6.3 厘米大小的肿瘤,并进行了针吸活检。骨内肿瘤由浆细胞弥漫性增生组成,细胞核偏位,有核周晕。通过免疫组化和原位杂交,肿瘤细胞 CD20-、CD3-、CD138+、κ+、λ-、EBER+,Ki67 标记指数约为 20%。进一步研究在血清中发现 IgG κ 单克隆蛋白,但无浆细胞肿瘤相关器官损害的证据,如高钙血症、贫血或肾功能不全。在形态学和流式细胞术研究中未发现骨髓浆细胞瘤。因此,诊断为 EBV 阳性浆细胞瘤。患者接受局部放射治疗并达到完全缓解。免疫功能正常患者中 EBV 阳性浆细胞瘤罕见,应与另一种具有浆细胞表型和侵袭性临床病程的 EBV 阳性肿瘤——浆母细胞淋巴瘤仔细鉴别。该病例还提出了一个重要问题:“在诊断浆细胞肿瘤时何时进行 EBER 原位杂交?”,这促使进一步进行大型病例系列研究。