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。

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.

目的 分析总结12例原发性肺淋巴上皮瘤样癌(LELC)的临床病理特点。方法 回顾性分析12例肺LELC患者的临床病理资料。结果 12例肺LELC患者的组织学标本中,肿瘤细胞均明显表达HCK,原位杂交EBER阳性率为91.7%。全组中位生存期61.3个月,2年和5年生存率分别为84.6%和57.7%。结论 原发性肺LELC临床罕见,发病可能与EB病毒感染有关,经及时治疗有较好的预后。

Objective To investigate the pathological and clinical significance of 12 cases with primary Lymphoepithelioma-like carcinoma of the lung (LELC). Methods Retrospectively detectded and analyzed the clincalpathological feature of 12 cases of LELC of the lung. Results Histological study showed that tumor cells were significantly expressed HCK by immunohistochemistry staining and showed out 91.7% positive rate of EBER by situ hybridization. In this series, the median survival time was 61.3 months. The overall 2 and 5 year survival rates of the 12 cases were 84.6% and 57.7%. Conclusion Pulmonary LELC is very rare. It may be associated with Epstein-Barr virus infection, and has a batter prognosis after therapy.