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1 型神经纤维瘤病中恶性外周神经鞘瘤的影像学标志物。

Imaging biomarkers for malignant peripheral nerve sheath tumors in neurofibromatosis type 1.

机构信息

From The Johns Hopkins University School of Medicine, Baltimore, MD.

出版信息

Neurology. 2019 Sep 10;93(11):e1076-e1084. doi: 10.1212/WNL.0000000000008092. Epub 2019 Aug 8.

DOI:10.1212/WNL.0000000000008092
PMID:31395668
Abstract

OBJECTIVE

To determine the utility of quantitative metrics obtained from fMRI using diffusion-weighted imaging (DWI)/apparent diffusion coefficient (ADC) mapping compared with metabolic (F-fluorodeoxyglucose [FDG]-PET/CT) imaging in patients with neurofibromatosis type 1 (NF1) for the characterization of peripheral nerve sheath tumors (PNSTs) as benign or malignant.

METHODS

This Institutional Review Board-approved, Health Insurance Portability and Accountability Act-compliant study retrospectively reviewed imaging of 55 PNSTs in 21 patients with NF1. Imaging included anatomic (unenhanced T1, fluid-sensitive, contrast-enhanced T1-weighted), functional DWI (b = 50, 400, 800 s/mm) and ADC mapping, magnetic resonance sequences, and FDG-PET/CT imaging. Anatomic (size), functional (minimum ADC values), and metabolic (maximum standardized uptake values [SUVmax]) imaging characteristics were recorded. ADC values were correlated with SUVmax. With histologic correlation for all malignant PNSTs (MPNSTs) or clinical or imaging stability (>12 months) for benign lesions used as reference standards, diagnostic accuracy was calculated.

RESULTS

Of 55 PNSTs, there were 19 (35%) malignant and 36 (65%) benign PNSTs. Benign PNSTs were overall smaller than MPNSTs (largest diameter 4.3 ± 1.3 vs 8.2 ± 3.3 cm, respectively, = 0.014). Benign PNSTs had higher ADCmin (×10 mm/s) than MPNSTs (1.6 ± 0.4 vs 0.6 ± 0.2, respectively, < 0.0001) and lower SUVmax than MPNSTs (3.2 ± 1.8 vs 8 ± 3.9, < 0.0001, respectively). ADCmin correlated inversely with SUVmax (correlation coefficient = -0.0.58, < 0.0001). Maintaining a sensitivity of 100% with threshold values of ADCmin ≤1 or SUVmax >3.2, DWI yielded a specificity of 94% while FDG-PET/CT offered a specificity of 83%.

CONCLUSIONS

Both quantitative metabolic imaging and functional imaging offer high sensitivity for the characterization of PNSTs in NF1; however, DWI/ADC mapping offers increased specificity and may be a more useful modality.

CLASSIFICATION OF EVIDENCE

This study provides Class II evidence that for patients with NF1, MRI using DWI/ADC mapping accurately distinguishes malignant and benign PNSTs.

摘要

目的

确定使用磁共振弥散加权成像(DWI)/表观弥散系数(ADC)映射获得的功能磁共振成像(fMRI)定量指标在神经纤维瘤病 1 型(NF1)患者外周神经鞘肿瘤(PNST)特征中的效用,以区分良性或恶性肿瘤。

方法

本研究经机构审查委员会批准,符合《健康保险流通与责任法案》的规定,回顾性分析了 21 例 NF1 患者 55 个 PNST 的影像学资料。影像学包括解剖学(未增强 T1、液体敏感、对比增强 T1 加权)、功能 DWI(b 值为 50、400、800 s/mm)和 ADC 图、磁共振序列和 F-氟脱氧葡萄糖(FDG)-正电子发射断层扫描/计算机断层扫描(PET/CT)成像。记录解剖学(大小)、功能(最小 ADC 值)和代谢(最大标准化摄取值 [SUVmax])影像学特征。分析 ADC 值与 SUVmax 的相关性。以所有恶性外周神经鞘瘤(MPNST)的组织学相关性或良性病变的临床或影像学稳定性(>12 个月)作为参考标准,计算诊断准确性。

结果

在 55 个 PNST 中,有 19 个(35%)为恶性,36 个(65%)为良性。良性 PNST 总体上比 MPNST 小(最大直径分别为 4.3±1.3cm 和 8.2±3.3cm, = 0.014)。良性 PNST 的 ADCmin(×10mm/s)高于 MPNST(分别为 1.6±0.4 和 0.6±0.2, < 0.0001),SUVmax 低于 MPNST(分别为 3.2±1.8 和 8±3.9, < 0.0001)。ADCmin 与 SUVmax 呈负相关(相关系数 = -0.058, < 0.0001)。以 ADCmin≤1 或 SUVmax>3.2 为阈值,DWI 具有 100%的敏感性,特异性为 94%,而 FDG-PET/CT 的特异性为 83%。

结论

定量代谢成像和功能成像均能高度敏感地对 NF1 患者的 PNST 进行特征描述;然而,DWI/ADC 图提供了更高的特异性,可能是一种更有用的方式。

证据分类

本研究提供了 II 级证据,表明对于 NF1 患者,DWI/ADC 磁共振成像能准确区分恶性和良性 PNST。

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