摘要
目的 :探讨儿童外周型原始神经外胚叶瘤(peripheral primitive neuroectodermal tumor,p PNET)的临床特点及综合治疗的疗效。方法 :回顾性分析2002年1月—2013年1月天津医科大学肿瘤医院收治的经手术、化疗和放疗的36例p PNET患儿的临床和随访资料,分析近期疗效、复发情况及1、3和5年的总生存率。结果 :36例患儿在治疗结束时完全缓解30例,部分缓解2例,疾病稳定2例,疾病进展1例,死亡1例,治疗有效率为94.4%。至随访结束时,14例患儿出现肿瘤复发,复发率为46.7%;无瘤生存16例,带瘤生存4例,死于肿瘤复发12例,死于肿瘤进展4例,中位生存期29个月。全组1、3和5年的总生存率分别为94.4%、50.0%和30.6%。初诊时有转移的患者的中位生存期(10个月)短于未转移的患者(中位生存期为33个月),差异有统计学意义(P=0.001)。结论 :儿童p PNET患者的预后差,初诊时未转移的患儿预后相对较好,复发和发生远处转移的患儿预后较差。
Objective: To investigate the clinical features of children with peripheral primitive neuroectodermal tumor(p PNET) and the efficacy of comprehensive therapy in these patients. Methods: The medical records and the follow-up information of 36 children with p PNET were collected and reviewed. All these patients received surgery, chemotherapy and radiotherapy between January 2002 and January 2013 in Tianjin Medical University Cancer Institute and Hospital. The therapeutic effect, recurrence rate and 1-, 3- and 5-year overall survival rates were analyzed. Results: At the end of the comprehensive therapy, 30 cases achieved complete remission, 2 cases achieved partial remission, 2 cases were in stable disease, one case had progressive disease, and one died of p PNET; the response rate reached 94.4%. By the end of the follow up, 14(46.7%) cases had tumor recurrence; sixteen cases had a disease-free survival, 4 cases survived with tumor, while 12 cases died of recurrence and 4 cases died of tumor progression. The median survival time was 29 months. The 1-, 3- and 5-year overall survival rate of all 36 cases were 94.4%, 50.0% and 30.6%, respectively. The median survival time of cases who were initialy diagnosed of metastatic p PNET was poorer than that of the cases who were initialy diagnosed of p PNET without metastasis(10 months vs 33 months, P = 0.001). Conclusion: Children with p PNET have very poor prognosis. Those who are initialy diagnosed of p PNET without metastasis have a better clinical outcome, while those who develop the recurrence or metastasis have a poor prognosis.
出处
《肿瘤》
CAS
CSCD
北大核心
2014年第12期1152-1156,共5页
Tumor
关键词
神经外胚瘤
原始
肿瘤治疗方案
儿童
预后
Neuroectodermal tumors,primitive
Antineoplastic protocols
Child
Prognosis
