广州医药 ›› 2020, Vol. 51 ›› Issue (5): 62-65.DOI: 10.3969/j.issn.1000-8535.2020.05.014

• 论著 • 上一篇    下一篇

脾脏炎性假瘤样滤泡树突细胞肉瘤的临床病理分析

唐昭亮, 王康玮, 王小拍, 杜洪   

  1. 广州市第一人民医院病理科,华南理工大学附属第二医院病理科(广州 510180)
  • 收稿日期:2020-03-01 出版日期:2020-09-20 发布日期:2021-11-28

Clinicopathologic analysis of inflammatory pseudotumor-like follicular dendritic cell sarcoma of the spleen

TANG Zhaoliang, WANG Kangwei, WANG Xiaopai, DU Hong   

  1. Department of Pathology,Guangzhou First People's Hospital,The Second Affiliated Hospital of South China University of Technology,Guangzhou 510180,China
  • Received:2020-03-01 Online:2020-09-20 Published:2021-11-28

摘要: 目的 探讨脾脏炎性假瘤样滤泡树突细胞肉瘤( IPT-like FDCS )的临床病理学特征、诊断及其鉴别诊断。方法 对2例脾脏 IPT-like FDCS 病例进行临床、组织病理学、免疫组织化学及原位杂交特征的观察及总结,并复习国内外相关文献。结果 2例患者均以腹部不适入院,平均年龄66岁,影像学检查提示脾脏占位;镜下特点:肿瘤由圆形、卵圆形及梭形细胞组成,呈编织状、束状或席纹状排列,背景中见多量淋巴细胞、浆细胞及少许中性粒细胞混杂并伴灶性出血、坏死;免疫组化结果:肿瘤细胞不同程度地表达CD21、CD23滤泡树突细胞标记物,两例均不表达CD35,EBER(EBV-encoded RNA)原位杂交显示瘤细胞散在阳性。结论 脾脏IPT-like FDCS 是一种好发于老年女性的罕见的低度恶性肿瘤,与EB病毒感染有关,其生物学行为相对惰性,手术完整切除肿瘤是最佳治疗方式,合理选用免疫标记物、原位杂交检测结合组织病理学可帮助正确诊断。

关键词: 炎性假瘤样滤泡树突状细胞肉瘤, 脾脏, EBER, 原位杂交

Abstract: Objective To study the clinicopathologic features,diagnosis and the differential diagnosis of inflammatory pseudotumor-like follicular dendritic cell sarcoma. Methods We analyzed clinical features,histopathological,immunohistochemical results and in situ hybridization characteristics of two cases. Besides,to relevant literatures of domestic and aboard were also reviewed. Results Two patients were hospitalized for abdominal discomfort, their average age was 66. Imageological examination showed splenic space-occupying. The neoplasms were composed of round,oval and spindle cells and were arranged in whorls and a spiral or storiform growth pattern,mixed with abundant lymphocytes and plasma cells and a few neutrophils with focal bleeding and necrosis. Immunohistochemically,varying degrees, tumor cells showed the expression of at least one of the FDC markers,including CD21 and CD23 protein except with CD35,with scattered positive EBER in situ hybridization. Conclusion Inflammatory pseudotumor-like follicular dendritic cell sarcoma of the spleen is a rare low-grade malignant tumor associated with EBV infection,which is older female predominated and with a inert biological behavior.The best treatment of the tumor is to complete surgical removal.Using reasonable application of immunohistochemical markers, in situ hybridization combined with histopathology are helpful for correct diagnosis.

Key words: Inflammatory pseudotumor-like follicular dendritic cell sarcoma, Spleen, EBER EBV-encoded RNA, In situ hybridization