Favorable Outcome of High-grade Endometrial Stromal Sarcoma in an Adolescent.
J Pediatr Hematol Oncol
; 46(5): e334-e337, 2024 07 01.
Article
em En
| MEDLINE
| ID: mdl-38691023
ABSTRACT
High-grade endometrial stromal sarcoma is a rare and aggressive soft tissue tumor characterized by YWHAENUTM2A/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 YWHAENUTM2E 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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias do Endométrio
/
Sarcoma do Estroma Endometrial
Limite:
Adolescent
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Female
/
Humans
Idioma:
En
Ano de publicação:
2024
Tipo de documento:
Article