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

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

World Neurosurg. 2015 Dec 31;

Authors: Qi W, Deng X, Liu T, Hou Y, Yang C, Wu L, Fang J, Tong X, Yang J, Xu Y

Abstract
OBJECTIVE: Primary spinal primitive neuroectodermal tumors (PNETs) are extremely rare entities. The purpose of this study was to analyze the difference between primary spinal central PNETs (cPNETs) and peripheral PNETs (pPNETs) in clinical and imaging characteristics and outcomes.
METHODS: A consecutive series of 25 patients with primary spinal PNETs were enrolled. The diagnosis was cPNETs in 6 patients for negative CD99 expression and pPNETs in 19 patients for positive CD99 expression. Overall, gross total resection (GTR) was achieved in 12 patients, subtotal resection was conducted in 9 patients and partial resection was performed in 4 patients. Fourteen patients underwent postoperative chemotherapy and 16 patients underwent radiotherapy.
RESULTS: The age at diagnosis was significantly younger in the cPNET group (mean 12.8 years) than the pPNET group (mean 22.5 years) (p=0.040), and the two pathologies didn't show 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) could bring about a longer median survival time (MST). Among the different treatment modalities, patients who underwent GTR, chemotherapy and radiotherapy all together had the highest 1- (100.0%) and 2-year (71.4%) survival rates and the longest MST (32 months).
CONCLUSIONS: Patients with spinal cPNETs had a younger age compared to pPNETs. The prognosis of spinal cPNETs and pPNETs are poor and there was no significant difference. The most beneficial treatment modality is GTR combined with adjuvant radiotherapy and chemotherapy.

PMID: 26748171 [PubMed - as supplied by publisher]

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