脾脏复合性血管内皮瘤临床病理观察

Clinicopathological features of splenic composite hemangioendothelioma

目的探讨脾脏复合性血管内皮瘤的临床病理特点、免疫组化特征及鉴别诊断。方法对1例脾脏复合性血管内皮瘤进行常规病理组织学和免疫组化,并结合相关文献,探讨其临床病理特征。结果患者男性,50岁。因"体检发现脾脏占位2天"入院,行腹腔镜下脾脏切除术。剖开脾脏组织,重990克,大小15 cm×15 cm×13 cm,切面暗红色,部分区灰白色,放射瘢痕样,局灶区见相依排列的多个小囊肿,内容清亮液体。镜下肿瘤由良性、中间性和恶性血管成分混合组成,良性成分包括局限性淋巴管瘤和海绵状血管瘤,中间性成分为淋巴管内乳头状血管内皮瘤(Dabaska瘤),恶性成分包括上皮样血管内皮瘤及中分化血管肉瘤。免疫组化显示海绵状血管瘤,淋巴管内乳头状血管内皮瘤(Dabaska瘤),上皮样血管内皮瘤及血管肉瘤的瘤细胞CD34、CD31、ERG、FⅧ、FLI-1、局限性淋巴管瘤瘤细胞D2-40、FLI-1均(+)。患者术后随访22个月,肿瘤无复发或转移。结论脾脏复合性血管内皮瘤是一种十分罕见的肿瘤,确诊主要依赖病理形态学,免疫组化检查对病理诊断和鉴别诊断起辅助作用。该瘤属于交界性/低度恶性肿瘤,有复发倾向,偶有淋巴结及远处转移。

Objective To investigate the clinicopathological,immunohistochemical characteristics and differential diagnosis of splenic composite hemangioendothelioma.Methods Pathological and immunohistochemical features were examined one case of splenic composite hemangioendothelioma,combined with relevant literature.Results 50-year-old male was admitted in the Third Hospital Affiliated to Soochow University.Laparoscopic splenectomy showed that the spleen mass was 990 g with size 15 cm×15 cm×13 cm.Cut section was dark-red,some areas were off-white,with radiation scars.Focal area showed multiple small cysts,and the contents were clear fluid.Microscopically,the tumor consisted of a mixture of benign,intermediate,and malignant vasculature.The benign components include localized lymphangioma and cavernous hemangioma.The intermediate component is Dabaska tumor.The malignancy included epithelioid hemangioendothelioma and moderately differentiated angiosarcoma.The hemangioendothelioma,and angiosarcoma tumor cells were positive for CD34,CD31,ERG and FVIII.Localized lymphangioma cells were positive for FLI-1 and D2-40.The patients were followed up for 22 months without recurrence or metastasis.Conclusion Splenic composite hemangioendothelioma is a very rare tumor.The diagnosis depends mainly on pathological morphology.Immunohistochemical examination plays an important role in pathological diagnosis and differential diagnosis.Our case is a borderline/low-grade malignancy with a tendency to relapse,with occasional lymph nodes and distant metastases.