子宫原始神经外胚层瘤2例并文献复习

2 Cases of Primitive Neuroectodermal Tumors of the Uterine and Literature Review

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。

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 cytoreductive surgery and chemotherapy, But recurred after only 4 months. and died 20 months. another patient who received tumorectomy 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.