国内首例子宫原发性原始神经外胚叶肿瘤报告并文献复习

Primary Primitive Neuroectodermal Tumor of Uterus: First Case Report in China and Review of the Literature

迄今国外文献仅报道16例子宫原发性原始神经外胚叶肿瘤(pPNET),本文在国内首次报道1例经手术病理检查确诊的子宫原发性原始神经外胚叶肿瘤。患者因经期延长、经量增多3个月入院,入院后经相关检查诊断为子宫肌瘤并中度贫血,后经多次输注浓缩红细胞,血红蛋白升至85.9 g/L后,在全麻下行子宫肌瘤剜除术,术后病理检查诊断为子宫前壁下段PNET,转入妇科肿瘤病房,行子宫全切、右侧附件切除并盆腔淋巴结清扫术。术后诊断子宫前壁下段pPNETⅠb期,予化疗。随访2.5年无复发。本文通过对本例临床资料进行回顾性分析并复习相关文献,探讨pPNET相关特点及诊治情况,以提高临床医生对pPNET的认识。

Up to now,only 16 cases of primary primitive neuroectodermal tumor（named as pPNET） of uterus have been reported abroad.In this text,a case of pPNET of uterus definitely diagnosed by surgery and pathological detect was for the first time reported in China.The patient was hospitalized for prolonged menstruation and increased menstruation volume for 3 months.Upon admission,the myoma of uterus accompanied with moderate anemia was primarily diagnosed by the related examinations.After hemoglobin increased to 85.9g/L by several blood transfusions of concentrated red blood cells,the enucleating of the myoma of uterus was completed in the general anesthesia.PNET of anterior wall＇s lower segment of uterus was diagnosed by the post-operation pathological detection.The patient was moved to gynecology tumor ward and treated with hysterectomy,right affix resection and pelvic cavity lymphonode dissection.Because final post-operation diagnosis was PNET of anterior wall＇s lower segment of uterus andⅠb phase,the patient received another chemotherapy treatment.The patient had no recurrence after two a 30-month follow up.This text is to explore the relevant features and the conditions of diagnosis and treatment of pPNET in order to improve the clinician＇s cognition of pPNET through retrospective analysis of the clinical data of this special case and review of the relative literatures.