CAJ | 학술논문

目的：探讨子宫原始神经外胚层瘤的临床表现、病理特点、治疗方法及预后。方法对2例子宫原始神经外胚叶肿瘤患者的临床资料进行回顾性分析并复习相关文献进行总结。结果2例患者的年龄分别为17岁和44岁，均经病理诊断确诊，Homer-Wright 菊形团为其特异性病理形态学表现，CD99表达均呈阳性。1例患者为IV 期，接受了肿瘤细胞减灭术，首次治疗结束后4个月复发，再次术后仍未获得满意生存，仅存活20个月；另1例仅行肿瘤切除术，术后给予化疗4疗程，随访至今未提示复发及转移。结论子宫原始神经外胚叶肿瘤主要依赖于病理及免疫组化结果明确诊断；手术为其主要治疗手段，术后可辅以放化疗，其预后与分期有关。
목적：탐토자궁원시신경외배층류적림상표현、병리특점、치료방법급예후。방법대2례자궁원시신경외배협종류환자적림상자료진행회고성분석병복습상관문헌진행총결。결과2례환자적년령분별위17세화44세，균경병리진단학진，Homer-Wright 국형단위기특이성병리형태학표현，CD99표체균정양성。1례환자위IV 기，접수료종류세포감멸술，수차치료결속후4개월복발，재차술후잉미획득만의생존，부존활20개월；령1례부행종류절제술，술후급여화료4료정，수방지금미제시복발급전이。결론자궁원시신경외배협종류주요의뢰우병리급면역조화결과명학진단；수술위기주요치료수단，술후가보이방화료，기예후여분기유관。
Objective To investigate the clinical and pathological characteristics, treatment, and prognosis of Peripheral Primitive Neuroectodermal Tumors of the uterus. Methods 2 cases of primary Primitive Neuroectodermal Tumor of uterus were included in Hunan Provincial Tumor Hospital. A retrospective analysis was carried out to analyze the clinical characteristics, clinicopathological features, treatment and prognosis of these patients. Results 2 patients aged 17 and 44 years old respectively. The final diagnosis was established by the pathological diagnosis. Homer-Wright daisy-group was a specific pathologic feature of pPNET. Positive expression of the CD99 was observed in all cases.One case who was FIGO stages IVB received cytoreduc-tive surgery and chemotherapy, But recurred after only 4 months. and died 20 months . another patient who received tumorec-tomy was survived in 5 months of follow-up. Conclusion Peripheral primitive neuroectodermal tumor (PNET) of Uterus was a very rare disease. CD99 and Homer-Wright daisy-group were both specific markers for PNET. The therapy included surgery, chemotherapy and radiotherapy. The prognosis of the primitive neuroectodermal tumors was related to their stage. The clinical experiences about the primitive neuroectodermal tumors were still needed.