外周性原始神经外胚层瘤15例临床分析  

Clinical Features of Peripheral Primitive Neuroectodermal Tumors:a Report of 15 Cases

作　　者：冯艺[1] 周宇红[1] 

机构地区：[1]复旦大学附属中山医院分部肿瘤内科,上海200052

出　　处：《中国临床医学》2012年第5期539-541,共3页Chinese Journal of Clinical Medicine

摘　　要：目的:分析外周性原始神经外胚层瘤(peripheral primitive neuroectodermal tumors,pPNET)的特点、治疗及预后。方法:对2006年6月—2011年5月的15例pPNET患者的临床资料进行分析。其中男性7例,女性8例;中位年龄23岁(11～55岁)。原发部位:骨6例,软组织4例,胸壁1例(Askin瘤),纵隔1例,后腹膜1例,肾脏1例,头皮1例。所有病例均采用手术或放疗以及全身化疗。化疗方案有3药联合[异环磷酰胺/环磷酰胺+蒽环类药物(阿霉素/表阿霉素)+顺铂、异环磷酰胺+阿霉素+氮烯米胺、异环磷酰胺/环磷酰胺+阿霉素+长春新碱]或2药联合(异环磷酰胺+依托泊甙、异环磷酰胺+阿霉素)。结果:5例辅助化疗的患者均无复发(随访期6～42个月,中位随访时间21个月)。10例姑息化疗的患者中,完全缓解(complete response,CR)1例、部分缓解(partial response,PR)3例、疾病稳定(stable disease,SD)3例、疾病进展(progressivedisease,PD)3例(其中2例病死),总有效率(CR+PR+SD)达70%;3例PD患者疾病进展时间(time to progression,TTP)分别是2.4、7、8个月。所有病例中位生存期尚未达到。结论:pPNET是一种高度恶性和异质性的肿瘤,预后差,主要依靠病理学诊断;采用合理的综合治疗方案可延长患者生存期,VAC/IE交替方案治疗pPNET是有前景且安全性较好的化疗方案。Objective：To investigate the clinical features,diagnosis,treatment and prognosis of peripheral primitive neuroectodermal tumor （pPNET）. Methods.. From June 2006 to May 2011, 15 cases of pPNET have been reviewed. There were 7 males and 8 females, with a median age of 23 years （ranged from 11 to 55）. Six cases occurred primarily in bone, 4 cases in soft tis- sues, one case in chest wall （Askin＇s）, one case in mediastinum, one case in retroperitoneum, one case in kidney and one case in scalp. All the cases underwent multimodal therapy（surgery/radiotherapy/chemotherapy ）. The chemotherapy regimens in- cluded three drugs combination（ifosfamide or cyclophosphamide ＋ adriamycin or epirubicin ＋ cisplatin, ifosfamide ＋ adriamycin ＋ dacarbazine,ifosfamide or cyclophosphamide ＋ adriamycin ＋ vincristine） or two drugs combination（ifosfamide ＋ etoposide, ifosfamide ＋ adriamycin）. Results= As far as June 2011 , none of the 5 cases undergoing adjuvant chemotherapy had recurrence （the follow-up time was 6-42 months, the median follow-up time was 21 months）. Among 10 cases undergoing palliative chemo-therapy, one case was complete response（CR）, 3 cases were partial response（PR）, 3 cases were stable disease （SD）, 3 cases were progressive disease （PD） and two of them died. Overall reaction ratio （ORR） was 70%. The time to progression （TTP） of three progressed patients were 2.4, 7 and 8 months. The median follow-up time was 21 months. Only 2 patients died because of disease progression. The median overall survival had not been reached. Conclusions= pPNET is a type of high malignant and heterogeneous tumor. The diagnosis of pPNET depends on pathology. Reasonable multimodal therapeutic regimens can prolong the survival. Alternative administration of CAV/IE for pPNET maybe a promising and safe chemotherapy regimen.

关 键 词：外周性原始神经外胚层瘤 综合治疗 尤文氏肉瘤家族肿瘤 

分 类 号：R739.4[医药卫生—肿瘤]