滤泡树突细胞肉瘤的临床病理观察

Clinicopathological Features of Follicular Dendritic Cell Sarcoma

目的总结分析滤泡树突细胞肉瘤(FDCS)的临床病理特征和免疫组织化学特点,以及IgG和IgG4的免疫组织化学表达情况,以加深对FDCS的认识。方法回顾性分析北京协和医院2005年1月至2018年12月全部病理确诊为FDCS病例9例,通过光镜观察、免疫组织化学染色及EB病毒编码的小RNA原位杂交检测,并查阅临床相关病历信息进行回顾性研究。结果9例病例男女比例4∶5,年龄16~53岁,平均年龄(38.2±9.7)岁。临床表现为体检发现肿物、淋巴结肿大、皮疹和发热。9例肿瘤部位分别为淋巴结、腹膜后、肾上腺、颈部肿物、腋下肿物和肝脏。超声显示为囊性或囊实性肿块,边界清楚,肿瘤直径1.5~15.0 cm。镜下肿瘤细胞呈梭形,以实片状、席纹状排列为主,胞质丰富淡染,可见空泡状核和小核仁,核分裂像1~3个/10倍视野,5例可见坏死;免疫组织化学CD21(6/9)、CD35(6/9)、CD23(7/9)阳性表达。结论FDCS是一种少见的恶性肿瘤,易漏诊误诊,免疫组织化学CD21、CD35、CD23联合应用有助于诊断和鉴别诊断。透明血管型巨淋巴结增殖症可能为FDCS的前驱病变,FDCS中可以有少量IgG4阳性浆细胞。目前治疗以手术切除为主。

Objective To explore the clinicopathological and immunohistochemical characteristics of follicular dendritic cell sarcoma(FDCS)and the expressions of IgG and IgG4.Methods 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.Results 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).Conclusions 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.