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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 Birbeck 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。
Objective To investigate the clinicopathological features and immunophenotypes of follicular dendritic cell sarcoma and to improve the understanding and diagnosis of this tumor. METHODS: Light microscopy, electron microscopy, and immunohistochemical staining [EnVision method, selected antibodies: CK (AE1/AE3), S-100 protein, CD1a, CD21, CD23, CD35, CD34, CD68, vimentin, desmin, HMB45, p53] observation and clinical data of 5 cases of follicular dendritic cell sarcoma clinical pathological analysis. All 5 patients were followed up. Results There were 3 males and 2 females in the 5 patients with an average age of 37 years. The tumors are located in the head and neck lymph nodes. Microscopically, the tumor tissues were arranged in nests, bundles, or vortexes. The tumor cells were oval or spindle-shaped. The cytoplasm was rich in acidity. The nuclear oval or fat spindles were irregularly distributed in clusters. Multinucleated giant cells were scattered; nuclear chromatin was sparse, the nucleolus were small and clear, and the number of mitoses varied, sometimes showing obvious nuclear atypia. The tumor cells were positive for CD21, CD23, and CD35. A few tumor cells were positive for CD68, S-100, and CD1a, CD34, HMB45, and CK were all negative. Under electron microscope, the tumor cells had long, distinct villous cytoplasmic processes and characteristic bridge-like connections. No Birbeck particles were observed. Follow-up of 5 to 52 months (mean 26 months), no recurrence or recurrence and metastasis. Conclusion Follicular dendritic cell sarcoma is a rare malignant tumor with undefined prognosis. Correct diagnosis requires histopathologic, electron microscopy, and immunohistochemistry, and should be differentiated from Langerhans cell sarcoma, dendritic cell sarcoma, malignant fibrous histiocytoma, melanoma, and spindle cell carcinoma. .