Abstract：
High-grade endometrial stromal sarcoma is a rare and aggressive soft tissue tumor characterized by YWHAE::NUTM2A/B translocations, diagnosis at a median of 50-60 years, and a poor prognosis (overall survival 30%-40%). We describe a 16-year-old patient with high-grade endometrial stromal sarcoma and regional nodal and pulmonary metastases who is a long-term survivor after grossly complete tumor resection, intensive chemotherapy, and pelvic radiotherapy. We discovered a previously undescribed YWHAE::NUTM2E translocation in the tumor. Our patient\'s favorable outcome suggests that intensive multimodality therapy with curative intent is appropriate for young patients with high-grade endometrial stromal sarcoma and highlights the importance of fertility preservation.
摘要：
高级别子宫内膜间质肉瘤是一种罕见且侵袭性的软组织肿瘤，其特征为YWHAE::NUTM2A/B易位，诊断的中位数为50-60年，和不良预后（总生存率30％-40％）。我们描述了一名16岁的高级别子宫内膜间质肉瘤和区域淋巴结和肺转移患者，他是肿瘤完全切除后的长期幸存者。强化化疗,和盆腔放疗。我们在肿瘤中发现了以前未描述的YWHAE::NUTM2E易位。我们患者的良好结果提示，具有治愈性目的的强化多模式治疗适合于患有高级别子宫内膜间质肉瘤的年轻患者，并强调了保留生育能力的重要性。
