Abstract
Purpose: Cranial Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are rarely reported because of their extremely low incidence, and the current understanding of these tumors is poor. The purpose of this study was to illustrate the clinical, radiological, and pathological features of cranial ES/pPNETs and to discuss prognostic factors by survival analysis.Methods: A total of 31 patients who were pathologically diagnosed with cranial ES/pPNETs between 2000 and 2019 were enrolled in this study. To identify which parameters were associated with higher progression-free survival (PFS) and overall survival (OS) rates, univariate and multivariate analyses were performed.Results: The mean follow-up period was 24.8 months (range, 1–109 months). Eighteen (58.1%) patients had local recurrence and seven (22.6%) patients had distant metastasis. The results of the univariate analysis suggest that the extent of resection and adjuvant radiotherapy are potential prognostic factors for PFS and OS. Adjuvant chemotherapy was associated with OS (P = 0.027) but not with PFS (P = 0.053). The multivariate analysis revealed that the extent of resection and adjuvant radiotherapy were independent prognostic factors for both PFS and OS. In addition, metastasis was an adverse prognostic factor for OS.Conclusions: Surgical management plays a crucial role in the treatment of cranial ES/pPNETs, and gross total resection should be striven for whenever possible. Post-operative radiotherapy is highly recommended to improve PFS and OS. This study also confirms that metastasis is an adverse prognostic factor for cranial ES/pPNETs.
Highlights
Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is an undifferentiated malignant, small, round cell tumor that usually arises from long bones and soft tissues in the second decade of life [1,2,3]
The results suggest that the extent of resection and adjuvant radiotherapy were independent prognostic factors for both progression-free survival (PFS) and overall survival (OS)
We found no biased gender distribution, as opposed to previous studies that reported that cranial ES/pPNET occurred with a slight male predisposition [4, 9]
Summary
Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is an undifferentiated malignant, small, round cell tumor that usually arises from long bones and soft tissues in the second decade of life [1,2,3]. It rarely occurs in the skull and meningeal tissue; only ∼50 cases of cranial ES/pPNETs have been reported in the English literature [4]. Given the paucity of reported cases, the clinical, radiological, and pathological features of cranial ES/pPNETs are still unclear. Comprehensive studies on the clinical, radiological, and pathological features of cranial ES/pPNETs are warranted
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
