侯婕 赵瑛

[摘要] 通过分析既往文献报道，结合我院收治的1例脾血管肉瘤患者的临床资料，整理出脾血管肉瘤的临床病理特点及临床诊治经验，以提高脾血管肉瘤的疗效。由于脾血管肉瘤缺乏特异性症状，诊断主要依靠影像学检查，多数病变在影像学上酷似海绵状血管瘤，确诊主要依靠病理及免疫组化检查,提示血管分化的免疫组化检测指标，如CD34、CD31、SMA、Vim、F8、UEA-1、CDl05、VEGFR3等至少两项为阳性；提示组织细胞分化的免疫组化检测指标，如CD68、CD8和S100蛋白等至少有一项为阳性。本病首选的治疗方法是脾脏切除，术后是否需要放化疗目前尚未有统一意见。

[关键词] 原发性脾血管肉瘤；脾肿瘤；诊断；治疗

[中图分类号] R733.2   [文献标识码] B   [文章编号] 2095-0616（2014）08-138-03

Splenic hemangiosarcoma：Report of 1 cases

HOU Jie  ZHAO Ying

The First Hospital of Shanxi Medical University,Taiyuan 030001,China

[Abstract] Through the analysis of the literature, combined with the clinical data of 1 case of splenic angiosarcoma patients in our hospital, sorting out the clinical pathologic characteristics and clinical experience in diagnosis and treatment of splenic angiosarcoma, in order to improve the efficacy of splenic angiosarcoma. Splenic angiosarcoma due to the lack of specific symptoms, the diagnosis mainly depends on imaging examination, the majority of lesions on imaging resembles a cavernous hemangioma, diagnosis depends on pathological and immunohistochemical examination, immunohistochemical detection indicators suggest that vascular differentiation： Such as, CD34, CD31, SMA, Vim, F8, UEA-1, CDl05, VEGFR3 at least two as positive; immune group tip cell differentiation detection index： Such asCD68, CD8 and S100 protein in at least one positive. Method for treatment of this disease is the first choice of spleen resection, postoperative need chemotherapy is not yet a unified opinion.

[Key words] Primary splenic angiosarcoma; Splenic tumor; Diagnosis; Treatment

脾血管肉瘤（spleen angioscarcoma，SA）又称脾恶性血管内皮细胞瘤,1879年Langhans首次报道，是间叶细胞来源的高度恶性肿瘤，由脾血窦向内皮细胞分化而成。临床非常罕见,占脾脏恶性肿瘤的7％[1]。任何年龄均可发病，发病年龄为14个月～89岁，平均发病年龄为50～60岁[2-4]。两性均可发病，但男性发病率较高，男女比例约为4∶3。病因尚不明，多认为与放疗、化疗、接触过氧化物、二氧化钍和砷等有毒物质有关。恶性程度极高，预后极差。极易经血行转移，多数患者在确诊前已发生转移。Neuhauser等[3]报道常见转移部位以次为肝（89％）、肺（78％）、淋巴结（56％）、骨（44％），其他学者报道亦可向肾脏、肾上腺和脑等器官发生转移。即使患者术前检查未发现肿瘤转移而行脾脏切除，术后肿瘤转移的发生率仍很高。

脾血管肉瘤临床十分罕见，我院曾收治1例，现报道如下。……