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[滤泡树突状细胞肉瘤的临床病理特征]

[Clinicopathological Features of Follicular Dendritic Cell Sarcoma].

作者信息

Lu Yao, Liu Qiao-Ling, Lu Tao, Pang Jun-Yi, Huo Zhen

机构信息

Department of Pathology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China.

Department of Pathology,Beijing Chinese Medicine Hospital Pinggu Hospital,Beijing 101200,China.

出版信息

Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2020 Aug 30;42(4):504-512. doi: 10.3881/j.issn.1000-503X.11785.

Abstract

To explore the clinicopathological and immunohistochemical characteristics of follicular dendritic cell sarcoma(FDCS)and the expressions of IgG and IgG4. We retrospectively analyzed the clinicopathological and immunohistochemical data of 9 pathologically confirmed FDCS cases in Peking Union Medical College Hospital from January 2005 to December 2018.Immunohistochemical staining of IgG and IgG4 were performed,and Epstein-Barr virus(EBV)-encoded RNA(EBER)in situ hybridization were carried out. Nine cases of FDCS included 4 men and 5 women aged 16-53 years [mean(38.2±9.7)years].The clinical manifestations included masses,lymph node enlargement,rash,and fever.The tumors were located in lymph node,retroperitoneal region,adrenal gland,neck,axillary region,and liver,respectively.Ultrasound showed clear boundary cystic or solid mass with maximum diameters of 1.5-15.0 cm.Microscopically,the spindle tumor cells were arranged in solid and storiform patterns with abundant and slightly stained cytoplasm,vacuolated nuclei,and small nucleoli.The mitosis was 1-3/10 high power fields,and necrosis was found in 5 cases.Immunohistochemically,the tumor cells were positive for CD21(6/9),CD35(6/9),and CD23(7/9). FDCS is a rare malignant tumor,which is easy to be missed.The combination of CD21,CD35,and CD23 is helpful for diagnosis.Hyaline-vascular type Castleman's disease may be the precursor of FDCS,and there may be only a small number of IgG4-positive plasma cells in FDCS.Surgical resection remains the main treatment for FDCS.

摘要

探讨滤泡树突状细胞肉瘤(FDCS)的临床病理及免疫组化特征以及IgG和IgG4的表达情况。我们回顾性分析了2005年1月至2018年12月在北京协和医院经病理确诊的9例FDCS病例的临床病理及免疫组化资料。进行了IgG和IgG4的免疫组化染色,并开展了爱泼斯坦-巴尔病毒(EBV)编码RNA(EBER)原位杂交。9例FDCS病例中,男性4例,女性5例,年龄16 - 53岁[平均(38.2±9.7)岁]。临床表现包括肿块、淋巴结肿大、皮疹和发热。肿瘤分别位于淋巴结、腹膜后区域、肾上腺、颈部、腋窝和肝脏。超声显示边界清晰的囊性或实性肿块,最大直径为1.5 - 15.0 cm。镜下,梭形肿瘤细胞呈实性和束状排列,胞质丰富且染色浅,核呈空泡状,核仁小。有丝分裂为1 - 3/10高倍视野,5例发现坏死。免疫组化显示,肿瘤细胞CD21(6/9)、CD35(6/9)和CD23(7/9)呈阳性。FDCS是一种罕见的恶性肿瘤,容易漏诊。CD21、CD35和CD23联合有助于诊断。透明血管型Castleman病可能是FDCS的前驱病变,且FDCS中可能仅有少量IgG4阳性浆细胞。手术切除仍是FDCS的主要治疗方法。

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