Comparison of Primary Spinal Central and Peripheral Primitive Neuroectodermal Tumors in Clinical and Imaging Characteristics and Long-Term Outcome

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• 作者：Wei Qi¹ ; ³ ; Xiaofeng Deng¹ ; ³ ; Tie Liu¹ ; ³ ; Yingzhi Hou¹ ; ³ ; Chenlong Yang¹ ; ³ ; Liang Wu¹ ; ³ ; Jingyi Fang² ; ³ ; Xianzeng Tong¹ ; ³ ; Jun Yang¹ ; ³ ; Yulun Xu¹ ; ³ ; ^{xuhuxi@sina.com" class="auth_mail" title="E-mail the corresponding author}
• 关键词：Ewing sarcoma ; Microsurgery ; Multimodality therapy ; Primitive neuroectodermal tumors ; Spinal cord
• 刊名：World Neurosurgery
• 出版年：2016
• 出版时间：April 2016
• 年：2016
• 卷：88
• 期：Complete
• 页码：359-369
• 全文大小：9149 K

文摘

Primary spinal primitive neuroectodermal tumors are extremely rare entities. The purpose of this study was to analyze the differences in clinical and imaging characteristics and outcomes between primary spinal central PNETs (cPNETs) and peripheral PNETs (pPNETs).

Methods

There were 25 consecutive patients with primary spinal primitive neuroectodermal tumors enrolled. The diagnosis was cPNET in 6 patients with negative CD99 expression and pPNET in 19 patients with positive CD99 expression. Gross total resection (GTR) was achieved in 12 patients, subtotal resection was performed in 9 patients, and partial resection was performed in 4 patients. Postoperative chemotherapy was given to 14 patients, and radiotherapy was given to 16 patients.

Results

The age at diagnosis was significantly younger in the cPNET group (mean 12.8 years) compared with the pPNET group (mean 22.5 years) (P = 0.040); the 2 pathologies did not show a significant difference in prognosis. GTR (P = 0.041), radiotherapy (P = 0.008), and GTR with radiotherapy (P = 0.009) were significant factors leading to a higher 2-year survival rate. Kaplan-Meier analysis showed that radiotherapy (P < 0.001) and GTR with radiotherapy (P = 0.040) resulted in a longer median survival time. Patients who underwent GTR, chemotherapy, and radiotherapy all together had the highest 1-year (100.0%) and 2-year (71.4%) survival rates and the longest median survival time (32 months).

Conclusions

Patients with spinal cPNETs were younger compared with patients with pPNETs. The prognosis of spinal cPNETs and pPNETs was poor with no significant difference between the entities. The most beneficial treatment modality is GTR combined with adjuvant radiotherapy and chemotherapy.