Abstract
Purpose: Primary spinal Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare, and the current understanding of these tumors is poor. The authors aimed to illustrate the clinical characteristics of primary spinal ES/pPNETs and to discuss prognostic factors by survival analysis.Methods: A total of 40 patients who were pathologically diagnosed with primary spinal ES/pPNETs between 2000 and 2018 were enrolled in this study. Progression-free survival (PFS) and overall survival (OS) were estimated by the Kaplan–Meier method to identify potential prognostic factors. Factors of p ≤ 0.1 in the Log-rank tests were subjected to multivariate analysis by Cox regression analysis.Results: The mean follow-up period was 23.8 (range, 2–93) months, and 24 (60.0%) patients had local recurrence and 11 (27.5%) patients had distant metastasis. The 1-, 2-, and 5-year PFS rates were 57.7, 30.4, and 9.5%, respectively. The 1-, 2-, and 5-year OS rates were 74.8, 50.7, and 12.2%, respectively. The univariate analysis suggested that resection mode, postoperative Frankel score, adjuvant chemotherapy and adjuvant radiotherapy were potential prognostic factors for OS and PFS. However, after these factors were subjected to multivariate analyses, only adjuvant radiotherapy and resection mode remained as independent prognostic factors.Conclusions: Total en bloc resection can significantly improve PFS for primary spinal ES/pPNETs and adjuvant radiotherapy was a favorable factor for PFS. Total en bloc resection and adjuvant radiotherapy considerably improve OS for patients with primary spinal ES/pPNETs.
Highlights
Primary spinal Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors are regarded as undifferentiated malignant small round cell tumors, which mostly occur in long bones, flat bones, ribs, and soft tissue
To illustrate the surgical management and prognostic factors of primary spinal ES/peripheral primitive neuroectodermal tumors (pPNETs), we retrospectively reviewed all of the cases surgically treated and pathologically confirmed as primary spinal ES/pPNETs at our institution between 2000 and 2018
28 (70%) patients presented with varied degrees of limb weakness, 20 (50%) patients presented with pain, and eight (20%) patients presented with incontinence
Summary
Primary spinal Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are regarded as undifferentiated malignant small round cell tumors, which mostly occur in long bones, flat bones, ribs, and soft tissue. ES/pPNETs account for 6–8% of primary malignant bone tumors, and rarely affect intraspinal/vertebral deep mesenchymal/meningeal tissue [1,2,3]. Due to a lack of clinic symptoms and specific biomarkers at the early stages of primary spinal ES/pPNETs, most patients are not diagnosed until advanced stages, which concomitantly worsens outcomes. Because the tumor has an aggressive clinical course—with a high tendency for both local recurrence and distant metastasis—a timely and accurate preoperative diagnosis of primary spinal ES/pPNETs could provide useful information for surgical planning. The rarity of the disease makes its purported surgical management and prognostic factors controversial. Radiological, and pathological factors associated with longer progression-free survival (PFS) and overall survival (OS) were analyzed
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