Abstract
BackgroundExtraosseous plasmacytoma, so called extramedullary plasmacytoma (EMP) is relatively rare in China. The aim was investigate the clinicopathologic features of EMP and the role of Immunophenotype and genotype detection in diagnosis of EMP.MethodsThirty-two cases of EMP were investigated retrospectively by histopathology, immunophenotype, genotype and survival analysis.ResultsClinically, the mean age of the patients was 53.4. Most of the patients received no treatment after the diagnosis was established, and the prognosis was relatively poor. Histologically, in 40% of the cases, the neoplastic cells were grade II or III. The neoplastic cells expressed one or more PC associated antigens. The immunophenotype of EMP and inflammation of sinonasal regions with numerous PC infiltrations were compared and showed some difference in expression of CD45, CD27, CD44v6 and Bcl-2 as well. Ig light chain restriction was detected in 87.5% of the cases.Conclusionswe described 32 Chinese cases of EMP, compare with that reported in the literature, some differences are presented, including higher percentage of grade II and III cases, clinically inconsistent treatment and management as well as poor outcome of the disease.
Highlights
Extraosseous plasmacytoma, so called extramedullary plasmacytoma (EMP) is relatively rare in China
Extraosseous plasmacytomas, so called extramedullary plasmacytomas (EMP) is defined by the updated WHO classification (2008) as localized plasma cell neoplasms that arise in tissues other than bone [1]
All cases selected fever the recommended diagnostic criteria for solitary EMP compiled by Soutar R, et al [6], they are as follows: (1) single extramedullary mass of clonal plasma cells; (2) histologically normal marrow aspirate and trephine; (3) normal results in skeletal survey, including radiology of long bone; (4) no anemia, hypercalcaemia or renal impairment due to plasma cell dyscrasia; (5) absent or low serum or urinary level of monoclonal immunoglobulin
Summary
In contrast to the well differentiated EMP, the neoplastic cells of grade III often showed plasmablastic morphology exhibiting large nuclei with open chromatin and prominent nucleoli, mitotic figures were seen and tumor giant cells were observed in 4 cases. 50% of the cases, the tumor cells expressed CD79a. In two cases some of tumor cells gave a positive reaction to CD20 or CD19, respectively. The tumor cells expressed CD56 and Cyclin D1 protein in 6 cases each (18.8%).
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