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 探讨原发性脾脏肿瘤的诊断与治疗。方法 选取2006年1月—2014年12月期间收治的58例原发脾脏肿瘤患者进行临床观察。结果 脾脏良性肿瘤45例,其中脾血管瘤21例,脾囊肿11例,脾淋巴管瘤8例,脾脏窦岸细胞血管瘤3例,脾错构瘤1例,脾脏硬化性血管瘤样结节性转化1例;脾脏恶性肿瘤13例,其中脾脏B细胞淋巴瘤11例,脾脏霍奇金淋巴瘤及脾脏血管肉瘤各1例。B超和CT是主要的检查方法。全组行脾切除术51例,其中开腹脾切除术23例,腹腔镜脾切除术28例;部分脾切除术5例;脾切除+胰腺体尾部切除术2例。所有病例无手术并发症。术后随访9个月~9年,平均随访时间(43.8±21.3)个月,41例脾脏良性肿瘤患者预后良好,无复发和转移;11例脾脏恶性肿瘤患者的1、3、5年生存率分别为81.8%、63.6%和27.2%。结论 原发性脾脏肿瘤的诊断主要依靠临床表现和影像学检查。手术切除不仅是脾脏肿瘤一种有效的治疗手段,也是重要的确诊方法。脾脏局限性良性肿瘤可选择部分脾切除术以保留脾脏功能。早期手术及术后联合放化疗可改善脾脏恶性肿瘤的预后。

Objective To investigate the diagnosis and treatment of primary splenic tumor. Methods The clinical data of 58 patients with primary splenic tumor from January 2006 to December 2014 were retrospectively analyzed. Results 45 cases were benign splenic tumor, including 21 hemangioma, 11 splenic cyst, 8 lymphangioma, 3 littoral cell angioma, 1 hamartoma and 1 sclerosing angiomatoid nodular transformation of spleen. 13 cases were malignant splenic tumor, including 12 lymphoma and 1 angiosarcoma. Ultrasound and CT were the main diagnostic methods. 51 cases underwent splenectomy, including 23 open splenectomy and 28 laparoscopic splenectomy. 5 cases underwent partial splenectomy and 2 splenectomy combined distal pancreatectomy. There had no postoperative complications. The follow-up period was 9 months to 9 years. 41 cases with benign splenic tumor had no recurrence or metastasis. The 1-, 3-, 5-years survival rate were 81.8%, 63.6% and 27.2% respectively of 11 patients with malignant splenic tumor. Conclusion The diagnostic of primary splenic tumor mostly rely on clinical features and imagic examination. Surgical resection is not only an effective treatment, but also a confirmed diagnosis method for primary splenic tumor. Partial splenectomy is a reasonable procedure for local benign splenic tumor. Early surgery, combined adjuvant chemotherapy and radiotherapy are important for improving the prognosis of malignant splenic tumor.