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目的总结儿童颅外原始神经外胚叶肿瘤(PNET)的临床资料及远期疗效,以进一步了解PNET的临床特点,指导诊疗及评估预后。方法回顾性分析我院2008年1月-2010年6月,收治的5例颅外PNET患儿的临床资料,分析其临床表现、诊断、治疗方法及预后。结果根据临床表现、体征、病理检查、影像学检查等,5例患儿均确诊为PNET,按照北京儿童医院-2007-CCG7942-PNET改良方案,经过手术、化疗及放疗等多学科联合治疗,3例患儿完成全部治疗,停止治疗前评估均完全缓解,其中1例停止治疗已1年8个月,无复发;余2例患儿分别于停药后8个月和停药后2个月本病复发。另外2例患儿因治疗期间疾病进展,其中1例死亡,另1例采用姑息治疗。结论儿童颅外PNET是一种高度恶性肿瘤,该病诊治难度大,易复发,需多学科联合诊治。
Objective To summarize the clinical data and long-term efficacy of children with extracranial primitive neuroectodermal tumor (PNET) to further understand the clinical features of PNET, to guide the diagnosis and treatment of prognosis. Methods The clinical data of 5 children with extracranial PNET admitted from January 2008 to June 2010 in our hospital were analyzed retrospectively. The clinical manifestations, diagnosis, treatment and prognosis were analyzed. Results According to the clinical manifestations, physical signs, pathological examination and imaging examination, all the 5 cases were diagnosed as PNET. According to the Beijing Children’s Hospital -2007-CCG7942-PNET improvement program, combined with multidisciplinary treatment such as surgery, chemotherapy and radiotherapy, 3 Complete treatment of all cases of patients with complete remission before treatment were completely relieved, of which 1 patient stopped treatment has been 1 year and 8 months, no recurrence; the remaining 2 cases of children were discontinued 8 months and 2 months after withdrawal The disease recurrence. Another 2 patients died of disease during the course of treatment, and 1 patient died of palliative care. Conclusion Extracranial PNET in children is a highly malignant tumor. The diagnosis and treatment of this disease is very difficult and easy to relapse, requiring multidisciplinary joint diagnosis and treatment.