结外滤泡树突细胞肉瘤临床病理学分析

Clinicopathologic analysis of extranodal follicular dendritic cell sarcoma

目的:探讨结外滤泡树突细胞肉瘤(FDCS)的临床病理特点、免疫表型及鉴别诊断。方法:应用光镜和免疫组织化学方法对我院8例结外FDCS进行临床和病理学观察,随访5年。结果:8例结外FDCS患者中,男性5例,女性3例,年龄18-61岁,分别位于扁桃体、腹膜后、上颌窦、乙状结肠及肩背部等部位。结外FDCS细胞梭形、卵圆形,呈束状、漩涡状或弥漫片状排列。胞浆丰富嗜酸性,胞界不清,核呈圆形、卵圆形、梭形或不规则形,核仁小而明显,常为两个或多个核仁,亦可无核仁。染色质呈点彩状分布,细而均匀,核分裂象易见。间质中常有数量不等的成熟淋巴细胞、浆细胞、嗜酸性粒细胞浸润。8例结外FDCS行CD21、D2-40、CD35、CXCL-13、CKpan、S-100、fasin、EMA、Ki67、Vimentin免疫组化检测,其中6例CD21及CD35阳性表达,5例D2-40及CXCL-13阳性表达。随访8例,2例发生于腹膜后者死亡,3例复发,3例经手术切除或辅以放疗和化疗后无复发和转移。结论:FDCS是一种少见类型的低至中度恶性肿瘤,复发率较高,CD21、D2-40、CD35及CXCL-13的联合应用有助于其正确的诊断和鉴别诊断。

Objective：To study the clinical and pathological characteristics,immunophenotypes and differential diagnosis of extranodal follicular dendritic cell sarcoma.Methods：Eight cases of extranodal follicular dendritic cell sarcoma were investigated by light microscopy and immunohistochemical staining.All patients were followed up for five years.Results：Five cases were males and three were females.Ages were from 18 to 61 years old.Tumors located in tonsil,retroperitoneal,maxillary sinus,colon sigmoideum,shoulder and back region respectively.Microscopically,the cells were spindle or oval and arranged in whored,storiform or diffuse patterns.They had richly eosinophilic cytoplasm with round,oval,spindle or irregular nucleus.The cells contained double or multiple nucleoli and the nucleoli were small and conspicuous.Sometimes there were no nucleoli.The chromatin was distributed with stippled shape,fine and uniform and the mitotic figures were easily found.There were many or few mature lymphocytes,plasma cells and eosinophils scattered in the interstitium.The expression of CD21,D2-40,CD35,CXCL-13,CKpan,S-100,fasin,EMA,Ki67 and Vimentin were detected by immunohistochemical staining in 8 cases.CD21 and CD35 expressed positively in 6 cases,D2-40 and CXCL-13 expressed positively in 5 cases respectively.Two cases located in retroperitoneum died,three cases happened recurrence.The others were no recurrence and metastasis after surgical resection with adjuvant radiotherapy and chemotherapy.Conclusion：The FDCS is a low to moderate grade malignant tumor with high recurrence rate.CD21,D2-40,CD35 and CXCL-13 are employed in correct diagnosis and differential diagnosis.