作者报告4例骨原发性血管外皮细胞癌(HP),并结合文献的41例作了评论。本组男性3例,女性1例,年龄为19、43、49及90岁。病变分别在颅骨、骶髂区、椎体与髂骨。组织学Ⅰ级1例,Ⅱ级2例,Ⅱ～Ⅲ级1例。颅骨者经手术与放疗无转移生存7年;另2例经手术加放疗加化疗,1例生存5个月,1例有复发生存5个月;另1例仅活检者(Ⅰ级)生存5个月。作者复习文献,骨HP占原发性骨肿瘤0.08%,占原发性恶性骨肿瘤0.1%,占恶性血管性肿瘤11%。男女比为1.8∶1,年龄从12～90岁,峰值发病年龄为40～50岁。最常受侵部位为盆骨和下肢。临床症状主要疼痛、肿块。从25 The authors reported 4 cases of primary vascular epithelial cell carcinoma (HP) and reviewed the literature in combination with 41 cases. This group of 3 males and 1 female, aged 19, 43, 49 and 90 years old. The lesions were in the skull, sacral region, vertebral body and sacrum. Histological grade I was 1 case, grade II 2 cases, and grade II-III 1 case. Skulls survived surgery and radiotherapy without metastasis for 7 years; the other 2 cases received surgery plus radiotherapy plus chemotherapy, 1 case survived 5 months, 1 case had recurrence survival 5 months, and 1 case only biopsy (grade I) survived 5 Months. The authors reviewed the literature, bone HP accounted for 0.08% of primary bone tumors, accounting for 0.1% of primary malignant bone tumors, accounting for 11% of malignant vascular tumors. The male to female ratio was 1.8:1, the age ranged from 12 to 90 years, and the peak onset age was 40 to 50 years. The most frequently affected sites are the pelvis and lower limbs. The main clinical symptoms are pain and lumps. From 25