外周原始神经外胚叶肿瘤23例临床分析

Peripheral primitive neuroectodermal tumors: analysis of 23 cases

目的:探讨外周原始神经外胚叶肿瘤(pPNET)的临床特征,以指导诊疗和评估预后。方法:收集我院23例pPNET患者的临床资料进行回顾总结,分析其临床表现、诊断和治疗的方法,并随访其生存期。结果:男性13例,女性10例,平均年龄为27.3岁,好发于躯干、四肢和中轴软组织包括胸壁、脊柱旁和腹腔等,多以出现进行性增大的局部包块起病。实验室和影像学检查无特异性指标,依据病理学明确诊断,治疗以手术为主,辅以全身化疗及局部放疗。平均生存期为15.9个月,年龄<16岁、原发于腹腔、不规范治疗的患者预后差。结论:pPNET是一种多发于青少年的高度恶性肿瘤,依靠病理学诊断,尽早发现,及时手术,辅以放化疗可延长生存期。

Objective：To investigate the clinical features of peripheral primitive neuroectodermal tumor（pPNET） time. Methods：The medical records of 23 pPNETs were reviewed, including the clinical features, diagnosis, treatment and survial. Results： There were 13 males and 10 females, median age 27.3 years. Trunks,limbs and the soft tissues of central axis,such as chest wall,vertebral column and abdominal cavity were often the primary lesions with gradually increasing tumors. Diagnosis depended on pathology, and there were no specific indexes on laboratory or imageology examinations. Surgery was the most important treatment, combined with chemotherapy and radiotherapy. Mean survival time was 15.9 months. Patients who were younger than 16 years, localized on abdominal cavity or received unstandard treatment had a relatively poor prognosis. Conclusion：pPNET is a extremely malignant tumor which often happen to adolescents. Diagnosis depend on pathology. Surgery combined with chemotherapy and radiotherapy in time could prolong the survival time.