Trigeminal schwannoma

三叉神经鞘瘤
  • 文章类型: Journal Article
    背景:三叉神经鞘瘤(TS)是颅内肿瘤,可引起明显的脑干压迫。TS切除可能是具有挑战性的,因为新的神经系统和颅神经缺陷的风险,特别是大(≥3厘米)或巨大(≥4厘米)TSs。由于先前的手术系列包括各种尺寸的TS,我们在此介绍我们通过显微外科手术切除治疗大型和巨大TS的临床经验.
    方法:这是一个回顾性研究,2012-2023年接受显微外科手术治疗的大型或巨型TS成人患者的单外科医生病例系列。
    结果:7例患者接受了TSs的显微外科手术切除(1例,6个巨人;4个男性;平均年龄39±14岁)。肿瘤分类为M型(硬膜间隙中窝;1例,14%),ME型(中窝颅外延伸;3例,43%),MP型(中、后窝2例,29%),或MPE型(中/后颅窝和颅外间隙;1例,14%)。6例患者接受额颞入路治疗(一名患者在同一坐位中结合经乳突开颅术,另一名患者采用延迟的经上颌入路),1例患者采用眶额颞入路治疗。5例(2例几乎全部切除)获得了全部切除。5例患者术前面部麻木,术后立即出现面部麻木,包括两个有恶化或新症状的。在平均22个月的随访中,有两名患者(28%)出现了新的非三叉神经颅神经缺陷。总的来说,80%的术前面部麻木患者和83%的面部麻木患者在术后过程中出现改善或消退。所有术前或术后新出现的非三叉神经肿瘤相关颅神经缺陷(4/4)的患者在随访中都有改善或消退。一名患者经历了保守治疗的肿瘤复发。
    结论:大型或巨大TSs的显微手术切除可以降低发病率和良好的长期颅神经功能。
    BACKGROUND: Trigeminal schwannomas (TSs) are intracranial tumors that can cause significant brainstem compression. TS resection can be challenging because of the risk of new neurologic and cranial nerve deficits, especially with large (≥ 3 cm) or giant (≥ 4 cm) TSs. As prior surgical series include TSs of all sizes, we herein present our clinical experience treating large and giant TSs via microsurgical resection.
    METHODS: This was a retrospective, single-surgeon case series of adult patients with large or giant TSs treated with microsurgery in 2012-2023.
    RESULTS: Seven patients underwent microsurgical resection for TSs (1 large, 6 giant; 4 males; mean age 39 ± 14 years). Tumors were classified as type M (middle fossa in the interdural space; 1 case, 14%), type ME (middle fossa with extracranial extension; 3 cases, 43%), type MP (middle and posterior fossae; 2 cases, 29%), or type MPE (middle/posterior fossae and extracranial space; 1 case, 14%). Six patients were treated with a frontotemporal approach (combined with transmastoid craniotomy in the same sitting in one patient and a delayed transmaxillary approach in another), and one patient was treated using an orbitofrontotemporal approach. Gross total resection was achieved in 5 cases (2 near-total resections). Five patients had preoperative facial numbness, and 6 had immediate postoperative facial numbness, including two with worsened or new symptoms. Two patients (28%) demonstrated new non-trigeminal cranial nerve deficits over mean follow-up of 22 months. Overall, 80% of patients with preoperative facial numbness and 83% with facial numbness at any point experienced improvement or resolution during their postoperative course. All patients with preoperative or new postoperative non-trigeminal tumor-related cranial nerve deficits (4/4) experienced improvement or resolution on follow-up. One patient experienced tumor recurrence that has been managed conservatively.
    CONCLUSIONS: Microsurgical resection of large or giant TSs can be performed with low morbidity and excellent long-term cranial nerve function.
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  • 文章类型: Case Reports
    由三叉神经引起的恶性周围神经鞘瘤(MPNSTs)极为罕见(仅45例,包括本案,已发表),并且据报道从周围神经鞘从头发展而不是从神经鞘瘤或神经纤维瘤转化。这里,我们报告了一例由三叉神经神经鞘瘤恶性转化引起的三叉神经MPNSTs,特别关注遗传因素。在对组织学上典型的良性神经鞘瘤进行几乎完全切除后,患者在初次切除10年后出现肿瘤再生。组织病理学和免疫化学检查证实复发性肿瘤是MPNST。综合基因组分析(基础一组基于基因分析)显示,只有复发的MPNST样本,不是神经鞘瘤的初步诊断,有基因突变,包括NF1-P。R2637*和TP53-p。Y234H,与恶性转化相关的候选基因突变。此外,逆转录聚合酶链反应结果表明,SH3PXD2A和HTRA1的融合已被报道为神经鞘瘤的主要遗传畸变之一,在复发性肿瘤中检测到。一起来看,我们可以说明通过神经鞘瘤从正常细胞发育MPNSTs的基因异常的积累过程。
    Malignant peripheral nerve sheath tumors (MPNSTs) arising from the trigeminal nerves are extremely rare (only 45 cases, including the present case, have been published) and have been reported to develop de novo from the peripheral nerve sheath and are not transformed from a schwannoma or neurofibroma. Here, we report a case of MPNSTs of the trigeminal nerve caused by the malignant transformation of a trigeminal schwannoma, with a particular focus on genetic considerations. After undergoing a near-total resection of a histologically typical benign schwannoma, the patient presented with regrowth of the tumor 10 years after the primary excision. Histopathologic and immunochemical examinations confirmed the recurrent tumor to be an MPNST. Comprehensive genomic analyses (FoundationOne panel-based gene assay) showed that only the recurrent MPNST sample, not the initial diagnosis of schwannoma, harbored genetic mutations, including NF1-p.R2637* and TP53-p.Y234H, candidate gene mutations associated with malignant transformation. Moreover, the results of reverse transcription polymerase chain reaction showed that the fusion of SH3PXD2A and HTRA1, which has been reported as one of the responsible genetic aberrations of schwannoma, was detected in the recurrent tumor. Taken together, we could illustrate the accumulation process of gene abnormalities for developing MPNSTs from normal cells via schwannomas.
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  • 文章类型: Case Reports
    颅内黑色素神经鞘瘤相当罕见,三叉神经的受累更罕见。早期诊断和手术切除是管理的支柱。这些肿瘤具有高复发倾向并且存在高转移可能性。由于预后不确定,因此应考虑辅助放疗。9个月前,一名23岁的男子开始在额头左侧出现麻木,进展到同侧脸颊。8个月前,患者开始向左侧看复视。他的亲属1个月前注意到他的声音有变化,右上下肢出现无力,这是逐渐进步的。患者有轻微吞咽困难。检查后,我们发现多个颅神经受累并伴有锥体束征.磁共振成像(MRI)提示左桥小脑角的轴外病变延伸到中颅窝,具有高的T1和T2信号损失,对比度增强。我们通过颞下硬膜外入路几乎完全切除了肿瘤。三叉神经黑色素神经鞘瘤是一种罕见的现象,由黑色素产生细胞和雪旺细胞组成。症状和体征的快速进展应提示怀疑病理可能的恶性性质。硬膜外颅底入路可降低术后缺陷的风险。将黑色素神经鞘瘤与恶性黑色素瘤区分开来在管理计划中至关重要。
    Intracranial melanotic schwannoma is quite rare, and involvement of the trigeminal nerve is even rarer. Early diagnosis and surgical excision are the mainstays of management. These tumors have a high tendency to recur and there is high possibility of metastasis. Adjuvant radiotherapy should be considered since the prognosis is uncertain. A 23-year-old man started developing numbness over the left side of the forehead 9 months ago that progressed to involve the ipsilateral cheek. The patient started having diplopia on looking to the left side 8 months ago. His relatives noticed a change in his voice 1 month ago and he developed weakness in the right upper and lower limbs, which was gradually progressive. The patient had slight difficulty swallowing. After examination, we found involvement of multiple cranial nerves with pyramidal signs. Magnetic resonance imaging (MRI) was suggestive of an extra-axial lesion in the left cerebellopontine angle extending into the middle cranial fossa, which was having high T1 and T2 signal loss with contrast enhancement. We achieved near-total excision of the tumor via a subtemporal extradural approach. Trigeminal melanotic schwannoma is a rare occurrence constituting melanin-producing cells and Schwann cells. Rapid progression of symptoms and signs should prompt the suspicion of the possible malignant nature of the pathology. Extradural skull base approaches reduce the risk of postoperative deficits. Differentiating melanotic schwannoma from malignant melanoma is of utmost importance in planning of management.
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  • 文章类型: Case Reports
    神经母细胞瘤是一种恶性的颅外肿瘤,常见于5岁以下的儿科人群,但在成人中很少见。只有少数原发性中枢神经系统神经母细胞瘤(PCN-NB)的病例被记录,大多数发生在年轻患者身上。在这篇文章中,我们报告了一个成人病例,在小脑桥脑角-中颅窝区域有PCN-NB,模仿另一个肿瘤。我们还讨论了PCN-NB的磁共振成像特征和病理特征以及鉴别诊断策略。
    Neuroblastoma is a malignant extra-cranial tumor that frequently arises in the pediatric population aged <5 years but is rare in adults. Only a few cases of primary central nervous system neuroblastoma (PCN-NB) have been documented, with most occurring in young patients. In this article, we report an adult case with a PCN-NB in the cerebellopontine angle-middle cranial fossa region that mimicked another neoplasm. We also discuss the magnetic resonance imaging features and pathological characteristics of PCN-NB and differential diagnosis strategies.
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  • 文章类型: Case Reports
    未经证实:三叉神经鞘瘤(TS)是头颈部区域非常罕见的肿瘤,并发生术中三叉神经心反射(TCR),手术期间,不应该被低估。这种罕见的脑干反射的生理功能尚未完全建立。
    未经评估:TCR发生在各种手术中,比如神经外科,颌面部,以心动过缓为前兆的牙科和颅底手术。
    UNASSIGNED:这是两名三叉神经神经鞘瘤患者的临床资料。
    未经批准:术中,在肿瘤解剖期间,两名患者均有心动过缓伴低血压.
    UNASSIGNED:第一个患者有自发恢复,但另一个患者需要血管加压药干预。
    UNASSIGNED:人们必须注意TCR的罕见发生,主要是在对罕见的TS进行操作时。无止境的术中监测和在操纵靠近神经的同时采取适当的措施将防止严重的并发症。
    UNASSIGNED: Trigeminal schwannoma (TS) is a very rare tumour in the head-and-neck region and the occurrence of intraoperative trigeminocardiac reflex (TCR), during surgery, should not be underestimated. The physiological function of this rare brainstem reflex is yet to be fully established.
    UNASSIGNED: TCR occurs in a variety of surgeries, such as neurosurgical, maxillofacial, dental and skull base surgeries with bradycardia as the presenting sign.
    UNASSIGNED: This is a clinical profile of two patients who presented with schwannoma of the trigeminal nerve.
    UNASSIGNED: Intraoperatively, during dissection of tumour, both patients had bradycardia with hypotension.
    UNASSIGNED: The first patient had a spontaneous recovery but the other required intervention with vasopressors.
    UNASSIGNED: One must be aware of the rare occurrence of TCR mainly while operating on a rare occurring TS. Ceaseless intraoperative monitoring and being prepared with adequate measures while manipulating close to the nerve will prevent serious complications.
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  • 文章类型: Case Reports
    目的:三叉神经鞘瘤是儿科人群中一种少见的肿瘤。文献中已经描述了几种手术方法。这项研究的目的是介绍一个11岁的男性,通过两阶段方法切除了巨大的哑铃形三叉神经鞘瘤,并回顾了儿童三叉神经鞘瘤的文献。
    方法:本研究为病例报告,包括术中录像和系统评价。根据系统评价和荟萃分析(PRISMA)指南的首选报告项目进行PubMed和Scopus搜索。
    结果:搜索策略总共产生了312个标题,其中13人被列入审查范围。这些描述的三叉神经鞘瘤病例,颅窝的临床表现和解剖排列高度可变。据报道,两阶段方法,然而,大多数研究描述了单阶段方法.术后常见的结果是第五神经的一系列紊乱。
    结论:根据肿瘤的构象,手术入路存在差异。然而,两阶段翼点颞下半坐位乙状窦后入路是安全的,在儿科患者中去除哑铃形三叉神经鞘瘤的实用有效策略。
    Trigeminal schwannoma is an uncommon tumor in pediatric patients. Several surgical approaches have been described in the literature.
    The case of an 11-year-old boy with a giant dumbbell-shaped trigeminal schwannoma removed through a 2-stage approach was presented with an intraoperative video. Using PubMed and Scopus, the literature on trigeminal schwannoma in pediatric patients was searched according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines.
    The search strategy yielded 312 titles, of which 13 were included in the review. Cases of trigeminal schwannoma were described, with a highly variable clinical presentation and anatomical arrangement in cranial fossae. Two-stage approaches were reported, although most studies described single-stage approaches. Common postoperative outcomes were a range of disturbances of cranial nerve V.
    The surgical approach varies based on the tumor conformation. However, a 2-stage pterional subtemporal and semisitting retrosigmoid approach is a safe, practical, and effective strategy for the removal of dumbbell-shaped trigeminal schwannoma in a pediatric patient.
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  • 文章类型: Journal Article
    未经证实:报告了几例脑膜瘤病例,这些脑膜瘤通过中、后颅窝的Meckel洞穴(MC)出现。然而,很少观察到相关的脑膜瘤具有哑铃形。
    方法:我们报告了一例罕见的病例,其中一名36岁的女性患有典型的哑铃状的MC脑膜瘤,磁共振成像(MRI)上的神经鞘瘤样表现,导致误导性的鉴别诊断。
    UNASSIGNED:在此案例报告中,我们讨论了在MRI上观察到的MC脑膜瘤的特征以及我们对这种情况的手术方法。
    结论:该肿瘤能够在临床和影像学上模拟三叉神经鞘瘤。此病例报告符合SCARE2020标准[1]。
    UNASSIGNED: Several meningioma cases arising through Meckel\'s cave (MC) at the middle and posterior fossa have been reported. However, few relevant meningiomas have been observed with a dumbbell shape.
    METHODS: We report a rare case of a 36-year-old woman with a meningioma of MC with a typical dumbbell-shaped, schwannoma-like presentation on magnetic resonance imaging (MRI), resulting in a misleading differential diagnosis.
    UNASSIGNED: In this case report, we discuss the characteristics of meningioma of MC observed on MRI and our surgical approach to this condition.
    CONCLUSIONS: This tumor was able to mimic a trigeminal schwannoma both clinically and radiographically. This case report has been reported in line with the SCARE 2020 criteria [1].
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  • 文章类型: Case Reports
    背景:三叉神经鞘瘤(TS)是一种罕见的组织学良性颅内病变,可累及第五脑神经的任何节段。鉴于其在诊断时通常令人印象深刻,并且经常累及颅底的关键神经血管结构,它代表了一个具有挑战性的实体治疗。儿科TS特别罕见,并提出了独特的挑战。同样,延伸到多个区室的肿瘤(例如,中颅窝,后颅窝,颅外间隙)众所周知难以手术治疗。通常需要联合或分阶段的手术方法来解决这些问题,以放射外科治疗为辅助手段。
    方法:作者介绍了一个9岁男孩的不寻常案例,涉及Meckel洞穴的复发性多室TS,小脑桥脑角,和颞下窝。使用额颞眶骨瓣切开术并结合硬膜间和硬膜外入路可实现几乎全部切除。
    结论:该病例报告增加了目前关于儿童多室TSs及其管理的文献。作者还提供了TS的简化分类,可以推广到其他颅底肿瘤。鉴于缺乏先例,作者打算增加关于这些罕见且具有挑战性的颅底病变的手术治疗的讨论.
    BACKGROUND: Trigeminal schwannoma (TS) is an uncommon and histologically benign intracranial lesion that can involve any segment of the fifth cranial nerve. Given its often impressive size at diagnosis and frequent involvement of critical neurovascular structures of the skull base, it represents a challenging entity to treat. Pediatric TS is particularly rare and presents unique challenges. Similarly, tumors with extension into multiple compartments (e.g., middle cranial fossa, posterior cranial fossa, extracranial spaces) are notoriously difficult to treat surgically. Combined or staged surgical approaches are typically required to address them, with radiosurgical treatment as an adjunct.
    METHODS: The authors presented the unusual case of a 9-year-old boy with a large, recurrent multicompartmental TS involving Meckel\'s cave, the cerebellopontine angle, and the infratemporal fossa. Near-total resection was achieved using a frontotemporal-orbitozygomatic craniotomy with a combined interdural and extradural approach.
    CONCLUSIONS: The case report adds to the current literature on multicompartmental TSs in children and their management. The authors also provided a simplified classification of TS that can be generalized to other skull base tumors. Given a lack of precedent, the authors intended to add to the discussion regarding surgical management of these rare and challenging skull base lesions.
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  • 文章类型: Case Reports
    Sarcoidosis is a systemic inflammatory condition causing increased immune system activity and manifesting as noncaseating granulomatous disease with the ability to affect multiple organ systems. Neurosarcoidosis is an uncommon presentation, with just 5-10% of patients with sarcoidosis experiencing intracranial disease. The diagnosis of neurosarcoidosis can be difficult, especially given the overlap of imaging findings with more common intracranial lesions. This case presents trigeminal neuralgia as the initial symptom of neurosarcoidosis and emphasizes the importance of a high clinical index of suspicion for neurosarcoidosis in patients with otherwise unexplained neurological symptoms.
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  • 文章类型: Case Reports
    BACKGROUND: Temporomandibular disorders (TMD) are diseases of the temporomandibular joint and masticatory muscles, and are often difficult to be diagnosed because they have various symptoms, pathological conditions and causes.
    METHODS: Herein, we report a 78-year-old male referred to our hospital with a diagnosis of TMD and presenting with facial asymmetry, marked deviation to the right side on vertical mandibular movement and complaints of abnormal perception at the right oral and buccal region. Past medical history revealed that he had undergone a right intracranial trigeminal schwannoma resection 9 years prior. Computed tomography (CT) showed disuse atrophy of the right side of 4 masticatory muscles and 2 suprahyoid muscles controlled by the motor component of the mandibular division (V3) of the trigeminal nerve (TGN). Together with the neurosurgeon, we confirmed that there was no recurrence of the tumor and explained to the patient that the oral and maxillofacial symptoms are after-effects of the operation, and we provided oral hygiene instructions and coordinated cleaning of the inside of the oral cavity.
    CONCLUSIONS: Although it is difficult to compare treatment methods from case to case, we believe that in our case, the patient\'s understanding of the cause of his discomfort contributed significantly to the improvement of his quality of life.
    CONCLUSIONS: We experienced a case of masticatory muscle disuse atrophy during long-term follow-up after resection of intracranial trigeminal schwannoma. Further studies are needed to develop the diagnostic and therapeutic protocols for disuse atrophy.
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