Trigeminal malignant peripheral nerve-sheath tumor: Systematic review, and case report treated by proton-beam therapy.

IF 1.5 4区医学 Q4 CLINICAL NEUROLOGY

Neurochirurgie Pub Date : 2024-12-10 DOI:10.1016/j.neuchi.2024.101621

Mathieu Lozouet, Lesueur Paul, Mazen Kallel, Gregoire Braux, Arthur Leclerc, Evelyne Emery

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引用次数: 0

Abstract

Background: Malignant peripheral nerve-sheath tumor (MPNST) is rare, occurring in approximately 0.001% of the general population. Cases involving cranial nerves, and particularly the trigeminal nerve (V), are exceptionally rare, with only 36 cases reported in a literature review in 2013. Standardized treatment and follow-up protocols are lacking. Here, we present a case of MPNST of the trigeminal nerve, provide a review of all cases described in the literature, and discuss treatment, surgical approach, recurrence rate, follow-up time and prognosis.

Methods: Based on PRISMA guidelines, we reviewed all cases of trigeminal MPNST published between 1950 and September 2023. We analyzed risk factors for recurrence. And we report the case of a patient with trigeminal MPNST admitted to our hospital.

Results: The literature review retrieved 39 relevant studies, reporting 48 cases. Patient age ranged from 4 to 71 years, for a mean 44.4 ± 17 years. The male:female ratio was 3.8:1. Most cases developed sporadically; 1 patient had neurofibromatosis type 1 and 1 had type 2. Surgery was the first-line treatment (85%), and radiotherapy was often included as adjuvant (65%). During a mean follow-up of 23.1 ± 24.4 months, fatal outcomes occurred in approximately 30% of patients. Only 1 case of systemic metastasis was reported. The only prognostic factor significantly associated with a lower rate of recurrence was complete surgical resection (p = 0.0035).

Conclusion: Cranial nerve MPNST is extremely rare, and trigeminal MPNST is even rarer. Treatment corresponds to current recommendations for intracranial MPNST, in which radical resection with adjuvant radiotherapy under stereotactic conditions results in the best outcome. Because of the high risk of recurrence, especially in case of incomplete resection, close follow-up is mandatory. Proton-beam therapy could be an interesting alternative adjuvant therapy after surgery.

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背景：恶性周围神经鞘瘤（MPNST）非常罕见，发病率约占总人口的 0.001%。涉及颅神经，尤其是三叉神经（V）的病例异常罕见，2013 年的文献综述中仅报告了 36 例。目前尚缺乏标准化的治疗和随访方案。在此，我们将介绍一例三叉神经多发性颅内压增高症（MPNST）病例，回顾文献中描述的所有病例，并讨论治疗、手术方法、复发率、随访时间和预后：根据 PRISMA 指南，我们回顾了 1950 年至 2023 年 9 月间发表的所有三叉神经 MPNST 病例。我们分析了复发的风险因素。并报告了本院收治的一例三叉神经间隙性脑肿瘤患者的病例：结果：文献综述共检索到 39 项相关研究，报告了 48 个病例。患者年龄从 4 岁到 71 岁不等，平均年龄为（44.4 ± 17）岁。男女比例为 3.8:1。大多数病例为散发性；1 名患者为神经纤维瘤病 1 型，1 名患者为 2 型。手术是一线治疗（85%），放疗通常作为辅助治疗（65%）。在平均 23.1 ± 24.4 个月的随访期间，约有 30% 的患者出现死亡。仅报告了一例全身转移病例。唯一与较低复发率显著相关的预后因素是完全手术切除（p = 0.0035）：结论：颅神经多发性坏死（MPNST）极为罕见，三叉神经多发性坏死（MPNST）更为罕见。治疗方法与目前针对颅内多发性骨髓营养不良症的建议一致，在立体定向条件下进行根治性切除并辅助放疗可获得最佳疗效。由于复发风险很高，尤其是在切除不彻底的情况下，因此必须进行密切随访。质子束疗法可以作为术后辅助治疗的一种有趣的选择。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neurochirurgie 医学-临床神经学

CiteScore

2.70

自引率

6.20%

发文量

100

审稿时长

29 days

期刊介绍： Neurochirurgie publishes articles on treatment, teaching and research, neurosurgery training and the professional aspects of our discipline, and also the history and progress of neurosurgery. It focuses on pathologies of the head, spine and central and peripheral nervous systems and their vascularization. All aspects of the specialty are dealt with: trauma, tumor, degenerative disease, infection, vascular pathology, and radiosurgery, and pediatrics. Transversal studies are also welcome: neuroanatomy, neurophysiology, neurology, neuropediatrics, psychiatry, neuropsychology, physical medicine and neurologic rehabilitation, neuro-anesthesia, neurologic intensive care, neuroradiology, functional exploration, neuropathology, neuro-ophthalmology, otoneurology, maxillofacial surgery, neuro-endocrinology and spine surgery. Technical and methodological aspects are also taken onboard: diagnostic and therapeutic techniques, methods for assessing results, epidemiology, surgical, interventional and radiological techniques, simulations and pathophysiological hypotheses, and educational tools. The editorial board may refuse submissions that fail to meet the journal''s aims and scope; such studies will not be peer-reviewed, and the editor in chief will promptly inform the corresponding author, so as not to delay submission to a more suitable journal. With a view to attracting an international audience of both readers and writers, Neurochirurgie especially welcomes articles in English, and gives priority to original studies. Other kinds of article - reviews, case reports, technical notes and meta-analyses - are equally published. Every year, a special edition is dedicated to the topic selected by the French Society of Neurosurgery for its annual report.