淋巴结指突状树突细胞肉瘤临床病理观察

Interdigitating dentritic cell sarcoma: a report of two cases with review of literature

目的探讨淋巴结原发性指突状树突细胞肉瘤的临床病理特点及鉴别诊断,提高对该肿瘤的诊断水平。方法对2例指突细胞肉瘤进行组织病理学、免疫组化及电镜观察。结果2例肿瘤均位于颈部淋巴结,光镜检查肿瘤组织呈席纹状、旋涡状或杂乱排列,瘤细胞卵圆形、梭形,胞质少,核卵圆形或短梭形,染色质细,少数有核仁,分裂象多见。瘤细胞S-100、CD68及vimentin(+),CD21、CD34、CK、CD45、SMA及HMB45均(-)。电镜下瘤细胞胞质有大量长指状突起,无桥粒连接及Birbeck颗粒。结论指突状树突细胞肉瘤是一种罕见的恶性肿瘤,预后差。诊断主要依靠电镜及免疫组化,并应与滤泡树突细胞肉瘤、朗格汉斯细胞肉瘤、恶性纤维组织细胞瘤、黑色素瘤、梭形细胞癌及其他肉瘤鉴别。

Objective To investigate the clinical pathological features and differential diagnosis of interdigitating dentrtic cell sarcoma （IDCS） to improve diagnostic level of it. Methods Two cases of IDCS were analyzed by light microscopy, electron microscopy, and immunohistochemistry. Results Two cases of IDCS were located in the cervical lymph node. Microscopically, the normal architectures were affaced by a proliferation of medium to large spindle-shaped cells with a fascicular, storiform or whorled patterns and with less cytoplasm. The mitotic figures was easily found. Immunohistochemical studies showed that the tumor cells was positive for S-100 protein, and CD68 but negative for CK, CD21, CD34, CD45, SMA and HMB45. Uhrastructurally, the tumor cells possessed complex interdigitating cytoplasmic dentritic processes, and cellular junction or Birbeck granules were absent. Conclusions IDCS is a rare malignant tumor and differential diagnosis includes Langerhans cell sarcoma, follicular dentritic cell sarcoma, malignant fibrous histocytoma, melanoma, metastatic spindle cell carcinoma and other sarcomas. Electron microscopy and immunohistochemistry are necssary for the diagnosis.