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目的总结腹膜后原始神经外胚层瘤/尤因肉瘤(PNET/EWS)的临床及组织病理特点,探讨其诊疗方法及预后情况。方法分析1例罕见的腹膜后PNET/EWS的临床特征、组织病理学特点以及诊断、治疗方法,同时复习近年来的国内外相关文献。结果患者当地医院行左肾切除+左腹膜后肿物部分切除,术后病理示PNET,并在当地医院行化疗。手术后4个月肿瘤复发,再次行手术切除并行化疗,1年后检查发现肿瘤再复发。结论腹膜后PNET/EWS是一种小圆形细胞组成的恶性肿瘤。诊断时应与其他腹腔和腹膜后软组织肿瘤相鉴别。治疗以手术切除为主,化疗效果不肯定。本病有侵袭性的临床过程,远处转移早,经常局部复发和区域淋巴结转移,肺、肝、骨和骨髓转移,预后较差。
Objective To summarize the clinical and histopathologic features of retroperitoneal primitive neuroectodermal/Ewing sarcoma (PNET/EWS), and to explore its diagnosis and treatment methods and prognosis. Methods The clinical features, histopathological features, diagnosis and treatment methods of a rare retroperitoneal PNET/EWS were analyzed. At the same time, the related domestic and foreign literatures were reviewed. Results The patient’s local hospital underwent left nephrectomy plus partial resection of the left retroperitoneal mass. Postoperative pathology showed PNET and chemotherapy was performed at the local hospital. After 4 months of surgery, the tumor recurred and surgical resection was performed concurrently. After 1 year, the tumor recurred. Conclusion Retroperitoneal PNET/EWS is a malignant tumor composed of small round cells. The diagnosis should be differentiated from other abdominal and retroperitoneal soft tissue tumors. The main treatment is surgical resection, and the effect of chemotherapy is not certain. The disease has an invasive clinical course, early distant metastasis, frequent local recurrence and lymph node metastasis, lung, liver, bone and bone marrow metastasis, and the prognosis is poor.