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内脏血管内皮肉瘤是罕见病，占内脏肿瘤不到１％，原发于脾脏者国内仅见一例报告。临床主要表现为左上腹痛、脾肿大、贫血等非特异性症状。病灶有多发性和多脏器的特点，可复发及转移，但病程趋于良性，平均生存期４９个月，５年存活率为３０％，１０年存活率为５％。手术前常不能确诊。组织病理学一般可分为分化程度较高型和未分化型。肿瘤细胞释放相关Ⅷ因子是其生物学特征，所以相关Ⅷ因子抗原反应（ＦⅧ－ＲＡＧ）及荆豆凝集素有诊断及鉴别诊断价值。电镜及影像学检查可提高确诊率。主要治疗是手术切除，而单纯放疗和化疗效果较差。
Visceral vascular endothelial sarcoma is a rare disease, accounting for less than 1% of visceral tumors, only one case of primary spleen in China. Clinical manifestations of left upper quadrant pain, splenomegaly, anemia and other non-specific symptoms. Lesions have multiple and multiple organ characteristics, recurrence and metastasis, but the course tends to be benign, the average survival of 49 months, 5-year survival rate was 30%, 10-year survival rate was 5%. Often can not be diagnosed before surgery. Histopathology can generally be divided into higher differentiation and undifferentiated type. Relevant Ⅷ factor related to the release of tumor cells is its biological characteristics, so the relevant factor Ⅷ factor antigen reaction (F Ⅷ-RAG) and the determination of the value of Vitexin lectin diagnosis. Electron microscopy and imaging examination can improve the diagnosis rate. The main treatment is surgical resection, and radiotherapy alone and chemotherapy less effective.