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目的 为了提高对骨原始神经外胚层肿瘤的临床及影像学认识。方法 收集 1978～ 2 0 0 2年 2 5年内国内文献报道的 12例及本院工作中遇到的 3例共 15例进行综合分析。结果 ①发病部位 :肱骨 6例 ,股骨 1例 ,髋骨 4例 ,椎体 2例 ,肩胛骨 1例 ,下颌骨 1例。②以青壮年多见 ,平均年龄为 2 6岁左右。女多于男 ,女 12例 ,男 3例。③临床发病缓慢 ,病程多为几个月 ,以局部疼痛为主要病症。④影像学表现 :以溶骨性骨质破坏及巨大软组织肿物为特征 ,其次针状骨膜增生或肿瘤骨也较常见。⑤病理以镜下见Homer -Wright菊形团为特点。结论 骨原始神经外胚层肿瘤好发于青壮年 ,女性多见 ,主要以溶骨性破坏为主 ,并有巨大软组织肿块 ,病理上有大量Homer -Wright菊形团。免疫组化NSF阳性 ,糖原染色阴性为主要特点。
Objective To improve the clinical and imaging knowledge of primitive neuroectodermal tumors. Methods A total of 12 cases reported in the domestic literature from 1978 to 2002 were collected and 15 cases were encountered in our work. Results ① The site of disease: 6 cases of humerus, 1 case of femur, 4 cases of hip, 2 cases of vertebral body, 1 case of scapula and 1 case of mandible. ② more common in young adults, the average age of about 26 years old. Female more than men and women in 12 cases, 3 males. ③ clinical onset of slow, mostly for several months duration, with local pain as the main symptom. ④ imaging findings: to osteolytic bone destruction and huge soft tissue mass is characterized by, followed by pericular periosteal hyperplasia or tumor bone is also more common. ⑤ pathology to see the mirror Homer-Wright daisy-shaped features. Conclusions Bone primitive neuroectodermal tumors usually occur in young and middle-aged women, and are more common in women. The majority of them are mainly osteolytic destruction, with massive soft tissue mass and large number of Homer-Wright chrysomlies in the pathology. Immunohistochemical NSF positive, negative glycogen staining as the main feature.