| Yu Liu,Weigang Zhao,Yusheng Shu. Peripheral primitive neuroectodermal tumor of the posterior mediastinum: a case report. Oncol Transl Med, 2014, 13: 441-443. |
| 后纵隔外周型原始神经外胚层肿瘤：一例病例报道 |
| Peripheral primitive neuroectodermal tumor of the posterior mediastinum: a case report |
| Received:July 04, 2014 Revised:July 26, 2014 |
| DOI：10.1007/s10330-014-1343-y |
| 中文关键词: 外周型原始神经外胚层肿瘤（pPNET）；神经源性纵隔肿瘤；后纵隔肿瘤；免疫组织化学 |
| 英文关键词: peripheral primitive neuroectodermal tumor (pPNET); neurogenic mediastinal tumor; posterior mediastinum tumor; immunohistochemistry |
| 基金项目: |
| Author Name | Affiliation | E-mail | | Yu Liu | Department of Cardiothoracic Surgery, Clinical Medicine College of Yangzhou University | 294393700@qq.com | | Weigang Zhao | Department of Cardiothoracic Surgery, Subei People’s Hospital, Yangzhou 225001, China | | | Yusheng Shu* | Department of Cardiothoracic Surgery, Subei People’s Hospital, Yangzhou 225001, China | shuyusheng65@163.com |
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| 中文摘要: |
| 外周型原始神经外胚层肿瘤是非常少见的恶性肿瘤，属于尤因肉瘤的一种，通常发生在儿童和青少年。我们报道一位41岁的此病患者，因右上腹疼痛入院，术前示后纵隔占位，手术切除活检可见小圆细胞，结合免疫组化结果诊断为外周型原始神经外胚层肿瘤。患者术后随访18个月未见明显复发迹象。结论：尽管外周型原始神经外胚层肿瘤是高度恶性肿瘤，病变的彻底切除以及术后辅助化疗能取得较满意的预后效果。 |
| 英文摘要: |
| Peripheral primitive neuroectodermal tumor (pPNET) is an extremely rare disease entity of malignant tumors belonging to the Ewing sarcoma family that usually occurs in children and adolescents. We describe a 41-year-old female who presented with right upper abdominal pain. Surgical resection and biopsy revealed small round-cell tumor. Combined with immunohistochemical analysis, pPNET was diagnosed. No evidence of recurrence was noted at 18 months postoperatively. Even thought pPNET is a highly malignant tumor, Wide tumor-free resection and multi-agent chemotherapy can also obtain good clinical outcomes. |
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