背景:恶性外周神经鞘瘤(MPNST)是一种极为罕见且侵袭性的肿瘤,关于其管理的文献有限。在这里,我们介绍了一系列手术管理的颅脑脊髓MPNSTs,分析他们的结果,并回顾文献。
方法:我们回顾性回顾了2005年1月至2023年5月在我们机构治疗的经手术管理的原发性颅脊髓MPNSTs。患者人口统计学,肿瘤特征,并评估治疗结果.使用Frankel等级和Karnofsky表现评分量化神经功能。描述性统计,秩和检验,进行了Kaplan-Meier生存分析.
结果:8例患者符合纳入标准(4例男性,4女)。演示时的中位年龄为38岁(范围15-67)。大多数肿瘤位于脊柱(75%),3例患者患有1型神经纤维瘤病。最常见的症状是感觉异常(50%)和视觉变化(13%)。中位肿瘤大小为3cm,大多数肿瘤为椭圆形(50%),边界清晰(75%)。六个肿瘤为高级别(75%),5例患者实现了大体全切除,其余3例患者进行次全切除。术后放化疗6例(75%),4例(50%),分别。5例(63%)发生局部复发,2例(25%)发生远处转移。中位总生存期为26.7个月。5例(63%)患者因复发死亡。
结论:原发性颅脊髓MPNSTs是罕见的,具有积极的临床过程。早期诊断和治疗对于治疗这些肿瘤至关重要。在这个单中心的小队列研究中,最大切除,低度病理学,年龄(<30岁),辅助放疗与生存率改善相关。
BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is an exceedingly rare and aggressive tumor, with limited literature on its management. Herein, we present our series of surgically managed craniospinal MPNSTs, analyze their outcomes, and
review the literature.
METHODS: We retrospectively reviewed surgically managed primary craniospinal MPNSTs treated at our institution between January 2005 and May 2023. Patient demographics, tumor features, and treatment outcomes were assessed. Neurological function was quantified using the Frankel grade and Karnofsky performance scores. Descriptive statistics, rank-sum tests, and Kaplan-Meier survival analyses were performed.
RESULTS: Eight patients satisfied the inclusion criteria (4 male, 4 female). The median age at presentation was 38 years (range 15-67). Most tumors were localized to the spine (75%), and 3 patients had neurofibromatosis type 1. The most common presenting symptoms were paresthesia (50%) and visual changes (13%). The median tumor size was 3 cm, and most tumors were oval-shaped (50%) with well-defined borders (75%). Six tumors were high grade (75%), and gross total resection was achieved in 5 patients, with subtotal resection in the remaining 3 patients. Postoperative radiotherapy and chemotherapy were performed in 6 (75%) and 4 (50%) cases, respectively. Local recurrence occurred in 5 (63%) cases, and distant metastases occurred in 2 (25%). The median overall survival was 26.7 months. Five (63%) patients died due to recurrence.
CONCLUSIONS: Primary craniospinal MPNSTs are rare and have an aggressive clinical course. Early diagnosis and treatment are essential for managing these tumors. In this single-center study with a small cohort, maximal resection, low-grade pathology, young age (< 30), and adjuvant radiotherapy were associated with improved survival.