摘要
目的探讨外周型原始神经外胚层肿瘤(pPNET)的临床表现、综合治疗效果及预后。方法回顾性分析34例pPNET患者的临床资料,其中肿瘤位于骨组织14例,胸壁6例,腹膜后和四肢软组织各3例,鼻腔鼻窦、椎管内和盆腔各2例,肾脏和肺内各1例。18例患者(包括1例新辅助化疗)行完全切除术,9例行部分切除术,其余7例仅行活检术以确诊,所有患者均接受了放化疗。结果截止于2011年6月,全组病例中有19例死亡,1、2年的总生存率分别为82%和41%。18例完全切除组的中位生存期和中位无疾病进展时间分别为24个月和12个月,16例非完全切除组分别为14个月和4个月,两组比较差异均有统计学意义(P<0.05)。结论 pPNET是一种易转移、易复发、侵袭性强的高度恶性肿瘤,采用合理的综合治疗方案可延长生存期。
Objective To study the clinical manifestations,effect of combined therapy and the prognostic factors of peripheral primitive neuroectodermal tumor（pPNET）.Methods The clinical characteristics of 34 patients with pPNET were analyzed retrospectively.Of the 34 cases,14 cases had bone invasion,6 cases had chest wall involvement,3 cases had retroperitoneal invasion and 3 cases had extremities soft tissue involvement.Among the remaining patients,2 cases had nasal cavity,vertebra and pelvic cavity invasion respectively,while 1 case had kidney and lung involvement respectively.Among them,18 cases underwent complete resection including 1 case received neoadjuvant chemotherapy,9 cases underwent partial resection and 7 cases received diagnostic biopsy.Then all the cases received local radiotherapy and chemotherapy.Results From follow up till date,19 patients died because of disease progression.The 1-,2-year overall survival rate was 82% and 41% respectively.The median overall survival and progress free survival of the complete resection group（18 patients） were 24 and 12 months respectively,while those in the non-complete resection group（16 patients） were 14 and 4 months.There was statistic significance between the two groups（P0.05）.Conclusion pPNET is a highly invasive and malignant disease with high potential for metastasis and recurrence.However,pPNET patients can have an improved overall survival if adequate and combined modality treatment is applied.
出处
《临床肿瘤学杂志》
CAS
2011年第10期887-890,共4页
Chinese Clinical Oncology
关键词
原始神经外胚层肿瘤
治疗
预后
Primitive neuroectodermal tumor
Treatment
Prognosis
