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中华妇幼临床医学杂志(电子版) ›› 2015, Vol. 11 ›› Issue (02) : 159 -163. doi: 10.3877/cma.j.issn.1673-5250.2015.02.004

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宫颈原始神经外胚叶肿瘤
雷呈志1,*,*()   
  1. 1. 100021 北京,中国医学科学院肿瘤医院妇科
  • 收稿日期:2015-02-28 出版日期:2015-04-01
  • 通信作者: 雷呈志

Primitive neuroectodermal tumors of the cervix uteri

Chengzhi Lei1()   

  1. 1. Department of Gynecology, Cancer Hospital Chinese Academy of Medical Sciences, Beijing 100021, China
  • Received:2015-02-28 Published:2015-04-01
  • Corresponding author: Chengzhi Lei
  • About author:
    Corresponding author: Lei Chengzhi, Email:
引用本文:

雷呈志. 宫颈原始神经外胚叶肿瘤[J/OL]. 中华妇幼临床医学杂志(电子版), 2015, 11(02): 159-163.

Chengzhi Lei. Primitive neuroectodermal tumors of the cervix uteri[J/OL]. Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition), 2015, 11(02): 159-163.

原始神经外胚叶肿瘤(PNET)是一类罕见的高度恶性肿瘤,多发生于儿童和青少年。世界卫生组织(WHO)将其分为中枢型PNET(cPNET)和外周型PNET(pPNET)。pPNET多发生于软组织、骨、腹膜后等部位,发生于宫颈的PNET十分罕见。目前文献报道的宫颈PNET病例很少,笔者拟通过复习文献,探讨宫颈PNET的临床病理学特点及其治疗与预后。宫颈PNET的临床诊治不同于宫颈鳞癌(SCC)和宫颈腺癌,目前对其主要采用化疗、手术和放疗结合的综合治疗措施。

Primary primitive neuroectodermal tumors (PNET) are rare and high-grade malignant tumors that mostly occur in children and young adults. PNET are divided into central-type PNET (cPNET) and peripheral-type PNET (pPNET) by the World Health Organization (WHO). pPNET majorly occur in soft tissues, skeleton, retroperitoneum and so on. pPNET of the cervix uteri is extremely rare. To date, few cases of pPNET of the cervix uteri have been reported in literature, and data on long term follow-up are not available. We focus on clinicopathologic profile, treatment, and clinical outcomes of patients with pPNET of cervix uteri by literatures review. Treatment strategies of pPNET of cervix uteri should be different from squamous carcinoma of the cervix (SCC) and adenocarcinoma of the cervix. Multimodality therapies including surgery, chemotherapy, radiotherapy and other treatment may prolong survival of patients of pPNET of cervix unteri.

表1 1987-2005年9例宫颈PNET情况比较
Table 1 Tumor and treatment characteristics of 9 patients with PNET of the cervix uteri: published in English literatures between 1987 and 2005
作者,文献报道时间/患者情况 治疗方案 结局
Russin等[4], 1987/60岁,G2P2,FIGO ⅠB2 腔内+外照射放疗,宫颈诊刮有残存肿瘤,行探查术+TAH+BSO,术中发现宫颈管内肿瘤和直肠、子宫窝处种植,术后行长春新碱+多柔比星+CTX化疗6个疗程 诊断后存活16个月
Sato等[5], 1996/44岁,G4P2, FIGO ⅠB2 TAH+左侧附件切除术+盆腔淋巴结切除术(附件和淋巴结无转移),术后顺铂+VP-16+多柔比星+CTX化疗(疗程不详) 术后存活6个月
Horn等[6], 1997/26岁,G2P2,FIGO ⅠB1 TAH+BSO+腔淋巴结清扫术(淋巴结未见转移)盆腔放疗。诊断后3年肺转移,行5-FU+顺铂化疗,胸部放疗 术后存活4.2年,死于肿瘤转移
Cenacchi等[7], 1998/36岁,G2P2,FIGO ⅠB2 TAH 术后存活18个月
Pauwels等[8], 2000/45岁,G2P2,FIGO ⅠB2 开腹子宫切除术,术后盆腔放疗 术后存活42个月
Tsao等[9], 2001/ 24岁,G3P2,FIGO ⅠB2,合并妊娠 术前肿瘤巨大,行术前化疗2个疗程,CTX+多柔比星+长春新碱1个疗程,IFO+VP-16化疗1个疗程。化疗后,TAH+卵巢悬吊术+腹主动脉旁取样,术后CTX+多柔比星+长春新碱化疗1个疗程,IFO+VP-16化疗1个疗程。术后盆腔放疗 存活期不详
Malpica等[10], 2002/35岁,FIGO ⅠB1 TAH+BSO+盆腔淋巴结清扫术+腹主动脉旁淋巴结取样。辅助化疗 诊断后存活5个月
Malpica等[10], 2002/51岁,FIGO ⅠB2 TAH+BSO+盆腔淋巴结清扫术+腹主动脉旁淋巴结取样。辅助化疗 诊断后存活18个月
Snijders-Keilholz等[11], 2005/21岁,G0P0 术前6个疗程化疗(多柔比星+IFO+VP-16)+行全子宫切除术。术后5个疗程化疗(长春新碱+IFO+放线菌素D) 诊断后存活27个月
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