Primary Ewing sarcoma/peripheral primitive neuroectodermal tumors in the cranial bone and mobile spine: what is the difference?

Jun Chen,Bo-Quan Qin,Jie Tan,Yu Wang,Shi-Zhou Wu,Hui Zhang,Qing-Yi Zhang

doi:10.1186/s12893-021-01452-4

Abstract

BackgroundPrimary Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are aggressive bone tumors that rarely occur in the axial skeleton, including the cranial bone and mobile spine. The purpose of this study was to investigate whether there were any differences in patient characteristics, treatment strategies, and outcomes between patients with ES/pPNETs of the cranial bone and those with ES/pPNETs of the mobile spine.MethodsA retrospective study was performed on 33 patients with ES/pPNETs who had been surgically treated and pathologically confirmed at our institution between 2010 and 2020. Patient characteristics were compared using Fisher exact tests or independent t tests. Survival rates were estimated via Kaplan–Meier survival analysis and compared using log-rank tests.ResultsThirteen patients had ES/pPNETs of the cranial bone (39.4%), while 20 patients had ES/pPNETs of the mobile spine (60.6%). Patients with ES/pPNETs of the cranial bone had a younger mean age (14.8 vs 22.6 years; p = 0.047) and longer mean disease duration (2.5 vs 1.9 months; p = 0.008) compared with those of patients with ES/pPNETs of the mobile spine. Kaplan–Meier analysis showed that gross total resection (GTR) and radiotherapy resulted in a longer median survival time. The overall survival rates and progression-free survival rates of patients with ES/pPNETs of the cranial bone versus those of the mobile spine were not significantly different (p = 0.386 and p = 0.368, respectively).ConclusionsPatients with ES/pPNETs of the cranial bone were younger compared to patients with ES/pPNETs of the mobile spine. There was no significant difference in the prognosis of patients with ES/pPNETs of the cranial bone versus those of the mobile spine. Taken together, our findings suggest that GTR and radiotherapy offer the best prognosis for improved long-term survival.

Highlights

Primary Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors are aggressive bone tumors that rarely occur in the axial skeleton, including the cranial bone and mobile spine
Most modern treatments—including combination surgery, postoperative radiotherapy, and chemotherapy—have improved the prognosis of primary ES/ peripheral primitive neuroectodermal tumors (pPNETs) over the past decades [8], the survival rate of patients with ES/pPNETs and unresectable disease remains poor, especially in those with large tumors occurring in the cranial bone or mobile spine [5, 9]
13 of the primary tumors arose in the cranial bone (39.4%) and 20 tumors arose in the mobile spine (60.6%)

Summary

Introduction

Primary Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are aggressive bone tumors that rarely occur in the axial skeleton, including the cranial bone and mobile spine. Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are the second most common primary sarcoma of bone [1]. Tumors located in the cranial bone and mobile spine account for approximately 1–4% and 8% of all primary sites, respectively [5, Chen et al BMC Surgery (2022) 22:4. Most modern treatments—including combination surgery, postoperative radiotherapy, and chemotherapy—have improved the prognosis of primary ES/ pPNETs over the past decades [8], the survival rate of patients with ES/pPNETs and unresectable disease remains poor, especially in those with large tumors occurring in the cranial bone or mobile spine [5, 9]. Some studies suggest that axial primary ES/pPNETs have a more aggressive phenotype due to the microenvironmental milieu of the axial skeleton [12, 13]

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