滤泡性树突状细胞肉瘤临床病理观察

目的探讨滤泡性树突状细胞肉瘤的临床病理特点及免疫表型,提高对该肿瘤的认识和诊断水平。方法通过光镜、电镜和免疫组织化学染色EnVision法,所选用抗体为：CK（AE1／AE3）、S-100蛋白、CD1a、CD21、CD23、CD35、CD34、CD68、波形蛋白、结蛋白、HMB45、p53]观察并结合临床资料对5例滤泡性树突状细胞肉瘤进行临床病理分析。5例均获随访。结果5例患者中男3例,女2例,平均年龄37岁。肿瘤均位于头颈部淋巴结。镜下观察：肿瘤组织呈片巢状、束状或旋涡状排列,瘤细胞卵圆形或梭形,胞质丰富淡嗜酸性;核卵圆形或胖梭形,趋向不规则成簇分布,散见多核巨细胞;核染色质稀疏,核仁小而清楚;核分裂象数目不等,有时显示明显的核异型。瘤细胞CD21、CD23、CD35阳性,少数瘤细胞CD68、S-100蛋白阳性,CD1a、CD34、HMB45、CK均阴性。电镜下瘤细胞有长而明显的绒毛状胞质突起及特征性的桥粒样连接,未见Birbeek颗粒。随访5～52个月（平均26个月）,无复发或再复发及转移。结论滤泡性树突状细胞肉瘤是一种少见的恶性肿瘤,预后不确定。正确诊断需要病理组织形态、电镜及免疫组织化学相结合,并应与朗格汉斯细胞肉瘤、指突状树突状细胞肉瘤、恶性纤维组织细胞瘤、黑色素瘤、梭形细胞癌等相鉴别。

Follicular dendritic cell sarcoma: a clinicopathologic study of five cases

Objective To study the clinical pathological features and immunophenotype of follicular dendritic cell sarcoma(FDCS)with discussion on its diagnostic clues to improve diagnostic level.Methods Five cases of FDCS were analyzed by clinical,pathologic and immunohistochemistry methods.Results Five cases of FDCS were located in the cervical lymph node.Microscopically,the normal architectures were affaced by ovoid,spindle-shaped with fascicular,diffuse or whorled patterns and with rich lightly eosinophilic cytoplasm,syncytial appearance.Nuclei tend to show irregular clustering,scattered multinucleated giant cell. Nucleoli often distinct,sometimes prominent.Mitotic count variable,may show significant cellular pleomorphism.Immunohistochemical studies show that the tumor cells were positive for CD21,CD35,but negative for CD1a,CD34,CK and HMB45.Under electron microscopy,the tumor cells possessed long villus cytoplasmic processes and desmosome-like junctions,Birbeck granules were absent.Conclusions FDCS is a rare malignant tumor and differential diagnosis includes Langerhans cell sarcoma,interdigitating dentric cell sarcoma,malignant fibrous histocytoma,melanoma,metastatic spindle cell carcinoma and others. Immunohistochemistry and electron microscopy are necessary for a correct diagnosis.