Lu Yao, Liu Qiao-Ling, Lu Tao, Pang Jun-Yi, Huo Zhen
Department of Pathology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China.
Department of Pathology,Beijing Chinese Medicine Hospital Pinggu Hospital,Beijing 101200,China.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2020 Aug 30;42(4):504-512. doi: 10.3881/j.issn.1000-503X.11785.
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.
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。