CAJ | 학술논문

目的探讨儿童骶尾部内胚窦瘤的治疗方法.方法回顾性分析天津市肿瘤医院儿童肿瘤科2000年1月至2011年1月收治的骶尾部内胚窦瘤患儿的临床病理资料.男4例,女10例,发病年龄10个月～10岁7个月.结果 1例Ⅰ期患儿仅行手术完整切除肿瘤及尾骨.10例先予PEB/HD-PEB方案化疗2～4个疗程后行肿瘤+尾骨切除术,化疗前穿刺活检病理均证实为内胚窦瘤,术后病理为内胚窦瘤或者恶性畸胎瘤化疗后改变,伴有坏死或退化变形细胞；术后继续PEB/HD-PEB方案化疗2～4个疗程.手术切除率为100％,术中均无直肠破裂.术后4例发生了切口感染.1例因放弃治疗而失访,1例Ⅳ期双肺转移的患儿因治疗无效死亡,其余12例均无瘤存活.结论骶尾部内胚窦瘤预后较好,手术+以铂类为基础的化疗是主要的治疗手段,术中完整切除尾骨是治疗的关键,否则易复发；新辅助化疗可有效提高手术切除率；新辅助化疗后再行手术的病例,即使术后病理结果未见恶性瘤细胞,也应继续按照化疗前病理诊断及分期完成相应的术后化疗,否则肿瘤易复发和转移.
목적 탐토인동저미부내배두류적치료방법.방법 회고성분석천진시종류의원인동종류과2000년1월지2011년1월수치적저미부내배두류환인적림상병리자료.남4례,녀10례,발병년령10개월～10세7개월.결과 1례Ⅰ기환인부행수술완정절제종류급미골.10례선여PEB/HD-PEB방안화료2～4개료정후행종류+미골절제술,화료전천자활검병리균증실위내배두류,술후병리위내배두류혹자악성기태류화료후개변,반유배사혹퇴화변형세포；술후계속PEB/HD-PEB방안화료2～4개료정.수술절제솔위100％,술중균무직장파렬.술후4례발생료절구감염.1례인방기치료이실방,1례Ⅳ기쌍폐전이적환인인치료무효사망,기여12례균무류존활.결론 저미부내배두류예후교호,수술+이박류위기출적화료시주요적치료수단,술중완정절제미골시치료적관건,부칙역복발；신보조화료가유효제고수술절제솔；신보조화료후재행수술적병례,즉사술후병리결과미견악성류세포,야응계속안조화료전병리진단급분기완성상응적술후화료,부칙종류역복발화전이.
Objective To evaluate the treatment of children with sacrococcygeal endodermal sinus tumor.Methods Clinical data of 14 cases of sacrococcygeal endoderrmal sinus tumor treated from January 2000 to January 2011 in this institute were reviewed retrospectively.There were four male and ten female cases,patients'age ranged from ten months to ten years and seven months.Results In all cases,ten patients who had been treated with preoperative chemotherapy received the resection of tumor and coccyx after completion of two to four cycles PEB /HD-PEB chemotherapy.The postoperative pathology confirmed the diagnosis of chemotherapy improved endodermal sinus tumor or malignant teratoma,accompanied by necrosis or degeneration of deformation of cells.Then the patients continued to PEB/HD-PEB chemotherapy regimen for 2-4 courses.One patient of stage Ⅰ was only underwent complete resection of the tumor and the coccyx.Operation resection rate was 100％,and no rupture of rectum occurred during the surgery Postoperative incision infection occurred in four cases.One patient was lost to follow-up,one patient died of chemotherapy resistance,the other twelve patients were still survived.Conclusions Patients with endodermal sinus tumor of the sacrococcygeal has a good prognosis.Operation with platinum-based chemotherapy is the main treatment.Complete resection of the coccyx is the key to avoid relapse.Neoadjuvant chemotherapy could improve the resection rate of operation.Even negative pathologically detection of malignant tumor cells,postoperative chemotherapy should continue and complete according to pathological diagnosis and staging of tumor before chemotherapy.