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Favorable Outcome of High-grade Endometrial Stromal Sarcoma in an Adolescent.
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Favorable Outcome of High-grade Endometrial Stromal Sarcoma in an Adolescent. Journal of pediatric hematology/oncology Hu, B., Howitt, B. E., Cizek, S., Diver, E., Hiniker, S., Crane, J., Daldrup-Link, H., Spunt, S. L. 2024Abstract
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.
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