Favorable Outcome of High-grade Endometrial Stromal Sarcoma in an Adolescent.
J Pediatr Hematol Oncol
; 46(5): e334-e337, 2024 07 01.
Article
in 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.
Full text:
1
Database:
MEDLINE
Main subject:
Endometrial Neoplasms
/
Sarcoma, Endometrial Stromal
Limits:
Adolescent
/
Female
/
Humans
Language:
En
Journal:
J Pediatr Hematol Oncol
Journal subject:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Year:
2024
Type:
Article