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Primary Ewing sarcoma/peripheral primitive neuroectodermal tumors in the cranial bone and mobile spine: what is the
Jun Chen1, Shi-Zhou Wu1, Jie Tan1
1Department of Orthopaedic Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan, 610041, People's Republic of China.
BMC Surgery
|January 8, 2022
Summary
Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) in the cranial bone occur in younger patients but have similar prognoses to those in the mobile spine. Gross total resection and radiotherapy improve survival for both groups.
Area of Science:
- Orthopedic Oncology
- Pediatric Oncology
- Skeletal Tumors
Background:
- Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are aggressive bone malignancies.
- These tumors rarely affect the axial skeleton, specifically cranial bone and mobile spine.
- Understanding differences in presentation and outcomes is crucial for targeted treatment.
Purpose of the Study:
- To compare patient characteristics, treatment strategies, and outcomes of cranial bone ES/pPNETs versus mobile spine ES/pPNETs.
- To identify potential differences in disease presentation and prognosis between these two locations.
- To evaluate the impact of treatment modalities on survival rates.
Main Methods:
- Retrospective analysis of 33 surgically treated and pathologically confirmed ES/pPNET patients (2010-2020).
- Comparison of patient demographics and disease characteristics using Fisher exact and independent t tests.
- Survival analysis utilizing Kaplan-Meier curves and log-rank tests.
Main Results:
- Cranial bone ES/pPNETs (39.4%) occurred in younger patients (mean age 14.8 years) with longer disease duration (2.5 months) compared to mobile spine ES/pPNETs (60.6%).
- Gross total resection (GTR) and radiotherapy correlated with longer median survival.
- No significant differences in overall or progression-free survival rates were observed between cranial bone and mobile spine ES/pPNETs.
Conclusions:
- Patients with cranial bone ES/pPNETs are generally younger than those with mobile spine ES/pPNETs.
- Prognosis for ES/pPNETs of the cranial bone is comparable to that of the mobile spine.
- GTR and radiotherapy are recommended for optimal long-term survival in patients with axial skeletal ES/pPNETs.
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