Cranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumors: A Retrospective Study Focused on Prognostic Factors and Long-Term Outcomes

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.

Primary Intracranial Ewing Sarcoma With EWSR1-FLI1 Gene Translocation Mimicking a Meningioma and a Multidisciplinary Therapeutic Approach: A Case Report and Systematic Review of Literatures

“…What differentiates this case from previous reports is that the response to chemotherapy only was prominent [ 1 3 4 5 23 ]. Adjuvant radiotherapy or chemotherapy or both for the treatment of ES/pPNET is known to be helpful in controlling ES/pPNET, especially when complete resection is not achieved.…”

“…Ewing sarcoma and peripheral primitive neuroectodermal tumor (ES/pPNET) is an undifferentiated malignant tumor with small and round cells radiologically mimicking meningioma [ 1 2 3 4 ]. ES/pPNET arises from soft tissues and long bones in children and young adults, whereas it is rarely reported in adults due to its extremely low incidence [ 1 5 6 ]. A meningioma is not always treated immediately but is rather subjected to a wait-and-see strategy.…”

Primary intracranial Ewing sarcoma in an infant

“…EWSR1::FLI1 fusion participates in dysregulating cell differentiation, proliferation, apoptosis, invasion, angiogenesis and metastasis 8. The treatment approach involves a multimodal regimen of surgery, chemotherapy and radiation 4 5 9. Our case adds to the clinical, neuroimaging, histopathological and molecular findings of a successfully treated primary intracranial ES in an infant without well-defined standard of care therapy.…”

A rare radiotherapy-sensitive primitive neuroectodermal tumor with APC gene amplification in an adult: a case report and literature review

“…Standard chemotherapy regimens consist of etoposide, vincristine, doxorubicin, and cyclophosphamide. Chen et al 22 examined 31 patients with Ewing's sarcoma of the skull and/or peripheral PNET and found a median PFS of 8 months and OS of 22 months; the OS rates at the 1-, 2-, and 5-year time points were 74.2%, 34.9%, and 13.1%, respectively. Additionally, surgery and radiotherapy can significantly improve the prognosis, especially for patients with intracranial and skeletal involvement.…”