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目的探讨炎性假瘤样滤泡树突状细胞肿瘤(IPT-like FDCT)的临床病理学特征,提高对该病的认识。方法报告5例炎性假瘤样滤泡树突状细胞肿瘤,结合文献分析其临床病理学特征、免疫表型及原位杂交检测EBV转录的核内小RNA(EBER)。结果5例炎性假瘤样滤泡树突状细胞肿瘤患者年龄35~78岁,平均52.6岁,其中女性2例,男性3例。3例肿瘤位于脾,1例位于肝,1例位于腹膜后。肿瘤通常与周围组织分界较清晰。镜下大量淋巴细胞、浆细胞背景中肿瘤细胞呈束状排列或散在分布;瘤细胞呈短梭形,胞质丰富,淡伊红染色,核呈梭形或卵圆形,空泡状,可见核仁,仅有轻度异型性。免疫表型:瘤细胞CD21、CD35及簇蛋白(clusterin)(+),EBER散在(+)。值得注意的是,其中3例伴随有IgG4阳性浆细胞的高表达。所有病例均经手术切除,随访2~96个月,其中1例失访,余4例均未出现复发和转移。结论炎性假瘤样滤泡树突状细胞肿瘤是一种罕见的低度恶性肿瘤,临床病理对其认识不足,易误诊,有必要加强对这种罕见疾病的认识。
Objective To investigate the clinicopathological features of inflammatory pseudotumor-like follicular dendritic cell tumor (IPT-like FDCT) and to improve its understanding of the disease. Methods Five cases of inflammatory pseudotumor-like follicular dendritic cell tumor were reported. The clinicopathological characteristics, immunophenotype and in situ hybridization were used to detect EBV-transcribed nuclear RNA (EBER). Results 5 cases of inflammatory pseudotumor follicular dendritic cell tumor patients aged 35 to 78 years, mean 52.6 years, of which 2 were women and 3 males. Three tumors were located in the spleen, one in the liver, and one in the retroperitoneum. Tumors are usually more clearly demarcated from the surrounding tissue. A large number of lymphocytes in the microscope, tumor cells in the plasma cell background were arranged in clusters or scattered; tumor cells were short fusiform, abundant cytoplasm, light Yi-red staining, nuclear fusiform or oval, vacuolar, visible Nucleolus, only mild atypia. Immunophenotype: tumor cells CD21, CD35 and clusterin (+), EBER scattered (+). It is noteworthy that 3 of them were accompanied by high expression of IgG4 positive plasma cells. All cases were surgically removed, followed up for 2 to 96 months, of which 1 case was lost, the remaining 4 cases were not recurrence and metastasis. Conclusions Inflammatory pseudotumor-like follicular dendritic cell tumor is a rare low-grade malignant tumor. Its clinicopathology is not well understood and easily misdiagnosed. Therefore, it is necessary to strengthen understanding of this rare disease.