神经内镜辅助下经乙状窦后入路显微手术切除大中型听神经瘤

目的 探讨神经内镜辅助下经乙状窦后入路显微手术切除大中型听神经瘤的效果。方法 回顾性分析2019年5月至2022年5月神经内镜联合显微镜经乙状窦后入路手术治疗的16例大中型听神经瘤的临床资料。结果 16例肿瘤均全切除,面神经解剖保留14例（87.5%）,术后面神经功能依据House-Brackmann分级Ⅰ级5例,Ⅱ级6例,Ⅲ级3例。术后无脑脊液漏及颅内感染。术后随访3~24个月,复查MRI增强未见肿瘤复发。结论 神经内镜辅助显微手术切除切除大中型听神经瘤,有助于减少手术创伤,减少脑脊液漏等并发症,保护面听神经功能,提高肿瘤全切除率。     

MR扩散加权成像对桥小脑角区实性神经鞘瘤与脑膜瘤的鉴别诊断价值

目的：探讨 MR扩散加权成像(DWI)及表观扩散系数(ADC)在桥小脑角区实性神经鞘瘤与脑膜瘤鉴别诊断中的价值。方法回顾性分析经术后病理证实的桥小脑角区12例实性神经鞘瘤和27例脑膜瘤的 DWI 图像,逐层勾画肿瘤边界以获得整体感兴趣区(ROI),得到肿瘤的平均 ADC 值(mADC)。同时以脑干 mADC 值为参考,计算得到肿瘤/脑干 ADC 比值(ADC ratio, rADC=肿瘤 mADC值/脑干 mADC值)。桥小脑角区实性神经鞘瘤和脑膜瘤之间的 mADC 和 rADC 值差异采用独立样本t检验分析。采用受试者工作特征曲线(ROC)分析 mADC 和 rADC 值在两者鉴别诊断中的价值。结果桥小脑角区实性神经鞘瘤mADC和 rADC值均明显高于脑膜瘤(mADC,P=0.001;rADC,P=0.002)。以 mADC=0.994×10-3 mm2/s 作为诊断阈值,鉴别实性神经鞘瘤和脑膜瘤的价值最优[曲线下面积(AUC)0.917;敏感度92%;特异度89%]。结论 MR DWI对桥小脑角区实性神经鞘瘤与脑膜瘤的鉴别诊断有一定的参考价值。     

前庭神经鞘膜瘤囊性变的治疗策略

摘要：目的分析前庭神经鞘膜瘤囊性变治疗策略的影响。方法回顾性分析2006年1月~2013年12月收治前庭神经鞘膜瘤697例,其中根据内听道及桥小脑角增强MRI发现为前庭神经鞘膜瘤束性变患者96例,同时从剩余601例中随机抽取96例作为实体肿瘤组,比较两组患者临床特性、术中特点和手术效果。结果前庭神经鞘膜瘤囊性变的临床进展明显较实性肿瘤快速,且听力症状较实性肿瘤患者严重,突发性耳聋率较高29（30.2%）,囊性肿瘤与面神经粘连较实体肿瘤严重,术后短期两组面神经功能差异无统计学意义（P>0.05）,但术后1年随访时CVS组面神经功能良好率明显低于SVS组（30.2% vs 44.8%,P=0.037）。两组在术后并发症发生率、死亡率、复发率方面无差异。结论前庭神经鞘膜瘤囊性变应首选手术切除,对于周围薄壁型囊性肿瘤,如剥离困难无法全切除,应以保留面神经功能为先,采取近全切除等措施,提高术后患者生活质量。     

An unusual case of glandular schwannoma

Glandular differentiation is exceedingly rare in peripheral nerve sheath tumors. In this report, an exceptional case of retroperitoneal glandular schwannoma is described in which the glandular element is markedly atypical, whereas the schwannian component is benign by morphologic analysis. To the best of our knowledge, the biologic behavior for such a lesion is unknown because similar cases have not yet been described in the literature.     

Papilledema secondary to vestibular schwannoma: An atypical case without intracranial hypertension

In most cases, vestibular schwannomas with papilledema are associated with intracranial hypertension secondary to hydrocephalus (obstructive or communicating). We describe the atypical case of a 39-years-old man who presented with bilateral papilledema revealing a vestibular schwannoma, but without hydrocephalus and with normal intracranial pressure. Ophtalmologic signs were completely resolved after tumor removal. The pathophysiological mechanism generally described to explain bilateral papilledema in such cases is tumor-induced hyperproteinorachia. However, in the absence of hydrocephalus or intracranial hypertension, this case raises the question of the mechanisms involved in the visual impairment related to vestibular schwannoma.     

Post-surgical vestibular schwannoma remnant tumors: What to do?

BackgroundVestibular schwannomas (VS) are benign tumors of the vestibular nerve's myelin sheath. The current trend in VS surgery is to preserve at the facial function, even if it means leaving a small vestibular schwannoma tumor remnant (VSTR) after the surgery. There is no defined therapeutic management VSTR. The aim of this study was to assess the evolution of the VSTR to define the best therapeutic management and identify predictive factors of VSTR progression.MethodsAmong the 256 patients treated surgically for VS in the Department of Neurosurgery at Angers University Hospital, 33 patients with a post-surgical VSTR were included in this retrospective study. For all surgical patients, the data collected were age at diagnosis, the Koos classification, the surgical access, the existence of a type 2 neurofibromatosis (NF2), the TR location and size on control MRI-scans. Patients had a bi-annual follow-up with clinical status and VSTR size assessment with MRI-scan. Survival analyzes were performed to determine the time and rate of VSTR progression, and identify factors of progression.ResultsThe mean follow-up of the population was 51 months. All VS remnant progression occurred between 38 and 58 months after surgery. In non-NF2 patients with first follow-up MRI-scan three months after surgery, 43% presented a spontaneous regression, 50% a stability and 7% a progression of the VSTR. In the same population with the 1-year MR-scan after surgery as baseline, 25% presented a spontaneous regression, 62.5% a stability and 12.5% a VSTR progression. These data are consistent with the data reported in the literature. The post-operative facial function impairment and an initial remnant ≥ 1.5 cm³ were found to be significant risk factors of VS remnant progression in non-NF2 population in univariate analysis (P = 0.048 and 0.031) but not in multivariate analysis.ConclusionIn our experience, the best therapeutic management of the post-surgical VSTP in non-NF2 patients with no risk factor of progression is a simple clinical radiological follow-up otherwise complementary radiosurgery should be considered.     

枕下-乙状窦后入路切除囊性听神经瘤

目的探讨囊性听神经瘤患者的流行病学、临床表现、治疗和预后。方法回顾性分析2000年1月至2010年6月收治的254例听神经瘤患者,对比研究囊性和实性听神经瘤患者在发病率、性别比例、肿瘤大小、临床表现、术后面听神经功能、术后其他并发症等的差异。结果囊性听神经瘤肿瘤体积更大,术后面听神经保留率更高,中枢感染率较高。结论大型囊性听神经瘤首选显微外科手术,残留肿瘤可采用立体定向放射神经外科处理。     

Malignant peripheral nerve sheath tumor of the vulva,an unusual differential diagnosis for vulvar mass

INTRODUCTION

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, up to one half of the MPNSTs occur in patients with neurofibromatosis type-1 (NF-1), while the rest are sporadic. Here, we present a 52-year-old woman with MPNST of the vulva without NF-1. We will discuss basics of the disease, treatment options and follow-up strategies.

PRESENTATION OF CASE

52-year-old female admitted to our hospital with complaint of abnormal uterine bleeding and rapidly growing vulvar mass. Excisional biopsy of the mass showed MPNST of the vulva. Afterwards, the patient underwent radical vulvectomy with inguinofemoral lymph node dissection. Short after the surgery, multiple lung metastasis were shown and responded to chemotherapy, but rapid local recurrence occurred short after the completion of the chemotherapy.

DISCUSSION

The primary treatment option in MPNSTs is surgical excision with or without adjuvant therapy. There is not enough data about the role of systemic chemotherapy in the management of MPNSTs and it still remains controversial.

CONCLUSION

In general, radiation therapy has not been demonstrated to improve overall survival. Complete surgical resection of the primary tumor is the mainstay of the treatment.     

面神经电生理监测下切除大型及巨大听神经瘤

目的探讨面神经电生理监测下切除大型及巨大听神经瘤技术。方法回顾分析22例大型及巨大听神经瘤临床资料,在面神经电生理监测下行显微手术切除,术后随访面神经功能。结果肿瘤全切除15例,次全切除7例。面神经自发肌电图在面神经受牵拉、挤压、生理盐水冲洗等操作过程中产生显著放电,刺激肌电图对寻找和辨认面神经具有重要作用。面神经位于肿瘤腹下方7例,腹上方2例,腹侧中部13例。所有病例面神经均解剖保留。术后随访6~42个月,面神经功能(House-Brackmann分级),H-B分级Ⅰ级11例,Ⅱ级5例,Ⅲ级2例,Ⅳ级4例。结论面神经电生理监测辅助显微手术对术中解剖与功能保留面神经具有重要价值。     

Endoscopic resection of benign esophageal schwannoma: Three case reports and review of literature

BACKGROUNDEsophageal schwannomas are uncommon esophageal submucosal benign tumors and are usually treated with surgery. CASE SUMMARYHere, we report three cases of middle/lower thoracic esophageal schwannoma treated successfully with endoscopic resection. These lesions were misdiagnosed as leiomyoma on preoperative imaging. During the endoscopic resection of such tumors, there is a risk of esophageal perforation due to their deep location. If possible, submucosal tunneling endoscopic resection should be used. CONCLUSIONFor larger schwannomas, endoscopy combined with thoracoscopy can be considered for en bloc resection. We performed a mini literature review in order to present the current status of diagnosis and treatment for esophageal schwannoma.