Safe marginal resection of atypical neurofibromas in neurofibromatosis type 1.
Safe marginal resection of atypical neurofibromas in neurofibromatosis type 1.
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DOI：
10.3171/2019.7.jns191353
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发表时间：
2020-11-01
影响因子：
4.1
通讯作者：
Chittiboina P
中科院分区：
文献类型：
--
作者：
Nelson CN;Dombi E;Rosenblum JS;Miettinen MM;Lehky TJ;Whitcomb PO;Hayes C;Scott G;Benzo S;Widemann BC;Chittiboina P
1型神经纤维瘤病（NF 1）患者易患内脏神经纤维瘤，其中一些可进展为癌前非典型神经纤维瘤（ANF）和恶性外周神经鞘瘤（MPNST）。虽然ANF的次全切除术可以防止恶性转化，从而防止死亡，没有神经并发症，局部复发需要再次手术。本研究的目的是评估通过术前连续容积MRI和18F-FDG-PET成像确定的靶向ANF结节边缘切除相关的手术发病率。作者分析了2008年至2018年期间在NIH临床中心治疗的11名NF 1患者的21个肿瘤的16个NF切除术的临床结局。通过Dunnett多重比较检验和t检验，测量靶病变的术前体积生长率和18F-FDG-PET SUVMax（肿瘤内最大标准化摄取值）以及载瘤神经的任何肌电图或神经传导速度异常，并与术后并发症、切除肿瘤的组织病理学分类和手术切缘一起进行评估。本文还介绍了安全切除ANF边缘的手术入路。11例连续NF 1患者（4例男性，7例女性;中位年龄18.5岁）接受了16次手术，对21个肿瘤进行了边缘切除。术前，14组连续MRI研究中的13组（93%）和11组18 F-FDG-PET扫描中的10组（91%）分别显示了所识别肿瘤的快速生长（每年体积增加≥ 20%）和亲和力（SUVMax ≥ 3.5）（中位肿瘤大小48.7 cm 3;中位生长率92%每年;中位SUVMax 6.45）。大多数手术（n = 14，88%）没有导致持续的术后载瘤神经相关并发症，迄今为止，没有切除的肿瘤复发。中位术后随访时间为2.45年（范围0.00-10.39年）。组织学分析证实ANF（6.51 ± 0.83，p = 0.0042）和低级别MPNST（13.8，p = 0.0001）的SUVMax显著高于良性神经纤维瘤（1.9）。本报告评价了连续成像（MRI和18 F-FDG-PET SUVMax）成功检测ANF的实用性，并证明了使用术中神经刺激和神经束显微解剖可以安全、保留神经束的囊外全切除ANF。
Patients with neurofibromatosis type 1 (NF1) are predisposed to visceral neurofibromas, some of which can progress to premalignant atypical neurofibromas (ANFs) and malignant peripheral nerve sheath tumors (MPNSTs). Though subtotal resection of ANF may prevent malignant transformation and thus deaths with no neural complications, local recurrences require reoperation. The aim of this study was to assess the surgical morbidity associated with marginal resection of targeted ANF nodules identified via preoperative serial volumetric MRI and 18F-FDG-PET imaging. The authors analyzed clinical outcomes of 16 NF resections of 21 tumors in 11 NF1 patients treated at the NIH Clinical Center between 2008 and 2018. Preoperative volumetric growth rates and 18F-FDG-PET SUVMax (maximum standardized uptake value within the tumor) of the target lesions and any electromyographic or nerve conduction velocity abnormalities of the parent nerves were measured and assessed in tandem with postoperative complications, histopathological classification of the resected tumors, and surgical margins through Dunnett’s multiple comparisons test and t-test. The surgical approach for safe marginal resection of ANF was also described. Eleven consecutive NF1 patients (4 male, 7 female; median age 18.5 years) underwent 16 surgical procedures for marginal resections of 21 tumors. Preoperatively, 13 of the 14 (93%) sets of serial MRI studies and 10 of the 11 (91%) 18F-FDG-PET scans showed rapid growth (≥ 20% increase in volume per year) and avidity (SUVMax ≥ 3.5) of the identified tumor, respectively (median tumor size 48.7 cm3; median growth rate 92% per year; median SUVMax 6.45). Most surgeries (n = 14, 88%) resulted in no persistent postoperative parent nerve–related complications, and to date, none of the resected tumors have recurred. The median length of postoperative follow-up has been 2.45 years (range 0.00–10.39 years). Histopathological analysis confirmed significantly greater SUVMax among the ANFs (6.51 ± 0.83, p = 0.0042) and low-grade MPNSTs (13.8, p = 0.0001) than in benign neurofibromas (1.9). This report evaluates the utility of serial imaging (MRI and 18F-FDG-PET SUVMax) to successfully detect ANF and demonstrates that safe, fascicle-sparing gross-total, extracapsular resection of ANF is possible with the use of intraoperative nerve stimulation and microdissection of nerve fascicles.