Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation
Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation
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DOI：
10.3171/2015.7.jns151056
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发表时间：
2016-11-01
影响因子：
4.1
通讯作者：
Link, Michael J.
中科院分区：
文献类型：
--
作者：
Carlson, Matthew L.;Jacob, Jeffrey T.;Link, Michael J.
目的第八脑神经恶性周围神经鞘膜瘤（MPNSTs）极为罕见.迄今为止，文献主要集中在假定为良性前庭神经鞘瘤（VS）的放射治疗后发生的MPNST，而没有事先照射产生的MPNST很少受到关注。本研究的目的是使用大型国家癌症登记数据库和英文文献的系统综述来描述流行病学、临床表现、病程和结局。此外，以前未报告的情况下presented.METHODS作者进行了分析的监测，流行病学和最终结果（SEER）数据库，文献的系统回顾，并提出一份病例报告。分析了SEER数据库中诊断为MPNST涉及第八CN，无既往放疗史的所有患者的数据。此外，对1980年1月至2015年3月期间英文文献中报告的所有病例进行了审查。最后，1个以前未报告的情况presented.RESULTS SEER登记确定了30例1992年和2012年之间。平均发病率为0.017/100万人/年（范围为0.000-0.0687/年）。诊断时的中位年龄为55岁，16例（53%）为女性。13例经尸检确诊。在17例活着时诊断的病例中，中位随访时间为118天，观察到3例死亡（18%）。当与良性VS的发生率相比，1041 VS的每1 MPNST产生的第八CN。包括作者中心以前未报告的病例，对英文文献的系统性综述产生了24份报告。诊断时的中位年龄为44岁，50%为女性，诊断时的中位肿瘤大小为3 cm。11名患者（46%）报告了VS典型的孤立性听前庭投诉，而13名患者（54%）表现出面部轻瘫或其他更具侵略性的过程的迹象。治疗包括单纯显微手术、显微手术加辅助放疗或显微手术加放化疗。接受治疗的患者中有61%复发，其中22%被诊断为脊柱转移瘤。最终，13名患者（54%）在诊断后中位3个月死于疾病进展。实现全切除的能力是唯一的功能，与改善疾病特异性survival.CONCLUSIONS MPNSTs的第八CN是非常罕见的，预示着预后不良。近一半的患者最初表现出与良性VS一致的结果，通常使早期诊断具有挑战性。根据这些数据，选择非手术治疗的患者应考虑早期放射学和临床随访，特别是症状持续时间短或表现不典型的患者。这些数据还提供了恶性肿瘤的基线发生率，在估计VS立体定向放射手术后的恶性转化风险时应考虑该基线发生率。
OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) of the eighth cranial nerve (CN) are exceedingly rare. To date the literature has focused on MPNSTs occurring after radiation therapy for presumed benign vestibular schwannomas (VSs), while MPNSTs arising without prior irradiation have received little attention. The objectives of the current study are to characterize the epidemiology, clinical presentation, disease course, and outcome using a large national cancer registry database and a systematic review of the English literature. Additionally, a previously unreported case is presented.METHODS The authors conducted an analysis of the Surveillance, Epidemiology, and End Results (SEER) database, a systematic review of the literature, and present a case report. Data from all patients identified in the SEER database with a diagnosis of MPNST involving the eighth CN, without a history of prior radiation, were analyzed. Additionally, all cases reported in the English literature between January 1980 and March 2015 were reviewed. Finally, 1 previously unreported case is presented.RESULTS The SEER registries identified 30 cases between 1992 and 2012. The average incidence was 0.017 per 1 million persons per year (range 0.000-0.0687 per year). The median age at diagnosis was 55 years, and 16 (53%) were women. Thirteen cases were diagnosed Upon autopsy. Of the 17 cases diagnosed while alive, the median follow-up was 118 days, with 3 deaths (18%) observed. When compared with the incidence of benign VS, 1041 VSs present for every 1 MPNST arising from the eighth CN. Including a previously unreported case from the authors' center, a systematic review of the English literature yielded 24 reports. The median age at diagnosis was 44 years, 50% were women, and the median tumor size at diagnosis was 3 cm. Eleven patients (46%) reported isolated audiovestibular complaints typical for VS while 13 (54%) exhibited facial paresis or other signs of a more aggressive process. Treatment included microsurgery alone, microsurgery with adjuvant radiation, or microsurgery with chemoradiation. Sixty-one percent of patients receiving treatment experienced recurrence, 22% of which were diagnosed with drop metastases to the spine. Ultimately, 13 patients (54%) died of progressive disease at a median of 3 months following diagnosis. The ability to achieve gross-total resection was the only feature that was associated with improved disease-specific survival.CONCLUSIONS MPNSTs of the eighth CN are extremely rare and portend a poor prognosis. Nearly half of patients initially present with findings consistent with a benign VS, often making an early diagnosis challenging. In light of these data, early radiological and clinical follow-up should be considered in those who elect nonoperative treatment, particularly in patients with a short duration of symptoms or atypical presentation. These data also provide a baseline rate of malignancy that should be considered when estimating the risk of malignant transformation following stereotactic radio surgery for VS.