脾脏原发性血管肉瘤临床病理观察

目的:探讨脾脏原发性血管肉瘤（primary angiosarcoma in the spleen,PAS）的临床病理学特征、免疫表型、诊断及鉴别诊断、治疗与预后，以提高对该肿瘤的认识。方法:对1例发生于脾脏的原发性血管肉瘤进行临床与病理学分析并复习相关文献，观察其组织学、免疫组化特征，探讨其临床病理学及预后。结果:患者女性，40岁。以“腹胀、腹痛、乏力1月余”入院。CT显示脾脏多发结节性占位。大体上脾脏正常结构完全消失，呈灰黄多结节状。镜下,肿瘤细胞形态多种多样。肿瘤大部分区域排列呈血窦样或裂隙样；部分区域血管腔相互吻合成复杂的海绵状、蜂窝状；部分区域可见不规则血管样结构沿血窦分布、突向血窦腔内，形成乳头簇结构突入管腔；另外可见分化较差的区域呈实性、梭形细胞结构。细胞异型性在不同区域表现不一致，大部分区域细胞异型性不明显，少部分区域衬覆细胞异型性明显，核分裂象多见（约9个/10 HPF）。肿瘤组织出血明显。免疫组化:肿瘤细胞弥漫表达ERG、CD31、CD34、Vimentin、FLI-1，不表达FⅧ、CD68、SMA、desmin，Ki-67增殖指数约30%。结论:脾脏原发性血管肉瘤非常罕见，但具有特异的组织学特征和免疫表型，需要与脾脏血管瘤、脾窦岸细胞血管瘤、脾窦岸细胞血管肉瘤等脾脏血管源性肿瘤相鉴别。该肿瘤易发生远处脏器转移，预后差，手术后需密切随诊。

Clinicopathologic features of primary angiosarcoma in the spleen

Objective:To investigate the clinicopathologic fetures,immunohistochemistry，diagnosis,differential diagnosis and prognosis of angiosarcoma in the spleen(PAS).Methods:One case of angiosarcoma in spleen was studied with histology,immunohistochemistry,followed by review of literature.Results:The patient was a 40-year old woman who was admitted to the hospital for abdominal distension and pain one month.CT revealed multiple nodules in the spleen.Macroscopically,normal structure of the spleen was replaced by grayish yellow nodule.Microscopically,the appearance was variable,like spleen-sinusoid,spongy,papillary and solid growth pattern.In some regions,the cells demonstrated marked cytologic atypia.Pathological mitotic figure was 9/10 HPF.The tumor cells were positive for ERG,CD31,CD34,Vimentin，FLI-1,but negative for FⅧ,CD68,SMA,desmin，and Ki-67 was about 30%.Conclusion:Primary angiosarcoma in the spleen is rare but with distinct histological and immunohischemical characteristics.It should be distinguished from other tumours of the spleen,for example,angioma，littoral cell angioma，littoral cell angiosarcoma.This neoplasm has poor prognosis and prone to distant metastasis.