Background Primary Ewing＇s sarcoma/primitive neuroectodermal tumor （ES/PNET） of urogenital tract is a rare condition with non-specific clinical presentations, which can make it difficult to diagnose. In this study,...Background Primary Ewing＇s sarcoma/primitive neuroectodermal tumor （ES/PNET） of urogenital tract is a rare condition with non-specific clinical presentations, which can make it difficult to diagnose. In this study, we summarize the clinical presentation, pathological features, therapeutic strategies, and prognosis of ES/PNET.展开更多
Peripheral primitive neuroectodermal tumor(PNET) of the kidney is a rare, aggressive tumor known for its recurrence and metastatic potential. Despite the frequency of venous extension to the renal veins and inferior v...Peripheral primitive neuroectodermal tumor(PNET) of the kidney is a rare, aggressive tumor known for its recurrence and metastatic potential. Despite the frequency of venous extension to the renal veins and inferior vena cava, pulmonary tumor embolism at the initial presentation is not common. We report a case of 22-year-old female with PNET of the kidney who presented with tumor embolism in the inferior vena cava(IVC) and bilateral pulmonary artery. The patient underwent surgical resection and histopathological analysis confirmed the presence of tumor within the IVC and pulmonary arteries. The patient received adjuvant chemotherapy and is currently doing well on follow-up.展开更多
背景和目的:尤文氏肉瘤家族肿瘤(Ewing's sarcoma family of tumor,ESFT)恶性度高、进展快,其最佳治疗方法目前仍在探讨中。本研究旨在分析ESFT的临床特点和探讨其治疗方法。方法:回顾性分析1995年1月至2008年4月中山大学肿瘤防治...背景和目的:尤文氏肉瘤家族肿瘤(Ewing's sarcoma family of tumor,ESFT)恶性度高、进展快,其最佳治疗方法目前仍在探讨中。本研究旨在分析ESFT的临床特点和探讨其治疗方法。方法:回顾性分析1995年1月至2008年4月中山大学肿瘤防治中心收治的92例初治ESFT。结果:骨尤文氏肉瘤(Ewing's sarcoma of bone,ETB)23例,骨外尤文氏肉瘤(extraosseous Ewing'ssarcoma,EOE)21例,外周性原始神经外胚叶瘤(peripheral primitive neuroectodermal tumor,PNET)43例,Askin瘤5例。中位随访时间31.5个月(10～137个月)。局限期综合治疗38例,单一治疗19例,两组3年生存(overall survival,OS)率分别为63%、20%,3年无事件生存(events-free survival,EFS)率分别为46%、18%,两组间生存差异具有统计学意义(P均<0.001)。局限期综合治疗患者在全身化疗的基础上加用手术加或不加放疗组远期生存均优于化疗+放疗组(χ2=7.591、9.212,P=0.006、0.002)。CAV/IE交替方案对局限期接受综合治疗患者延长了无事件生存期,但其总生存时间差异无统计学意义(χ2=6.950、3.530,P=0.008、0.06)。多因素生存分析显示治疗模式以及疗效是独立的预后因素。结论:综合治疗能明显改善局限期ESFT患者疗效和生存,手术加化疗加或不加放疗的治疗模式在疗效和生存方面优于化疗加放疗治疗模式。治疗模式和近期疗效是独立的预后因素。展开更多
文摘Background Primary Ewing＇s sarcoma/primitive neuroectodermal tumor （ES/PNET） of urogenital tract is a rare condition with non-specific clinical presentations, which can make it difficult to diagnose. In this study, we summarize the clinical presentation, pathological features, therapeutic strategies, and prognosis of ES/PNET.
文摘Peripheral primitive neuroectodermal tumor(PNET) of the kidney is a rare, aggressive tumor known for its recurrence and metastatic potential. Despite the frequency of venous extension to the renal veins and inferior vena cava, pulmonary tumor embolism at the initial presentation is not common. We report a case of 22-year-old female with PNET of the kidney who presented with tumor embolism in the inferior vena cava(IVC) and bilateral pulmonary artery. The patient underwent surgical resection and histopathological analysis confirmed the presence of tumor within the IVC and pulmonary arteries. The patient received adjuvant chemotherapy and is currently doing well on follow-up.
文摘背景和目的:尤文氏肉瘤家族肿瘤(Ewing's sarcoma family of tumor,ESFT)恶性度高、进展快,其最佳治疗方法目前仍在探讨中。本研究旨在分析ESFT的临床特点和探讨其治疗方法。方法:回顾性分析1995年1月至2008年4月中山大学肿瘤防治中心收治的92例初治ESFT。结果:骨尤文氏肉瘤(Ewing's sarcoma of bone,ETB)23例,骨外尤文氏肉瘤(extraosseous Ewing'ssarcoma,EOE)21例,外周性原始神经外胚叶瘤(peripheral primitive neuroectodermal tumor,PNET)43例,Askin瘤5例。中位随访时间31.5个月(10～137个月)。局限期综合治疗38例,单一治疗19例,两组3年生存(overall survival,OS)率分别为63%、20%,3年无事件生存(events-free survival,EFS)率分别为46%、18%,两组间生存差异具有统计学意义(P均<0.001)。局限期综合治疗患者在全身化疗的基础上加用手术加或不加放疗组远期生存均优于化疗+放疗组(χ2=7.591、9.212,P=0.006、0.002)。CAV/IE交替方案对局限期接受综合治疗患者延长了无事件生存期,但其总生存时间差异无统计学意义(χ2=6.950、3.530,P=0.008、0.06)。多因素生存分析显示治疗模式以及疗效是独立的预后因素。结论:综合治疗能明显改善局限期ESFT患者疗效和生存,手术加化疗加或不加放疗的治疗模式在疗效和生存方面优于化疗加放疗治疗模式。治疗模式和近期疗效是独立的预后因素。
