外周神经源肿瘤的超声诊断及鉴别诊断

目的探讨超声在外周神经源肿瘤的诊断及鉴别诊断中的应用价值。方法对8例经手术病理证实的外周神经源肿瘤的声像图进行回顾分析。结果神经鞘瘤和神经纤维瘤具有不同的声像图特征。结论超声检查可根据肿瘤与神经的关系鉴别神经纤维瘤和神经鞘瘤。     

神经纤维瘤的整形外科治疗

目的 研究神经纤维瘤及巨大神经纤维瘤的整形外科治疗。方法 回顾性分析1995年到2003年我院收治的10例神经纤维瘤的临床诊断及治疗。10例神经纤维瘤中，4例为孤立性神经纤维瘤，6例为神经纤维瘤病；其中2例为头面部巨大神经纤维瘤，5例为躯体及四肢巨大神经纤维瘤。结果 对头面部巨大神经纤维瘤进行肿瘤全切除，背阔肌肌皮瓣游离移植，修复皮肤缺损及面部整形；躯体及四肢巨大神经纤维瘤行肿瘤全切除，皮片移植修复，或部分切除直接缝合。结论 神经纤维瘤采用整形外科方法切除修复，具有切除较彻底，修复塑形好的优点。对于巨大神经纤维瘤，对术中出血应有充分准备。     

高频彩超鉴别诊断神经鞘瘤的意义与价值

目的评估高频彩超声图像特征对神经鞘瘤的鉴别诊断意义与价值。方法选取2010-07—2015-07我院外科手术切除的四肢或颈部肿瘤病灶患者157例,所有肿瘤病灶术前均行高频彩超检查。现对这些肿块的彩超图像进行整理,分析肿块的包膜、形态、血流、钙化及与神经的关系对鉴别诊断的价值。并计算高频彩超诊断神经鞘瘤的灵敏性、特异度及准确性。结果肿瘤病灶共157个,其中高频彩超拟诊为神经鞘瘤39例,病理证实33例,余118例病变超声提示非神经鞘瘤,病理证实16例为神经鞘瘤,高频彩超诊断神经鞘瘤的灵敏度、特异性、准确度分别为67.35%,94.44%,85.98%。其中肿块的包膜完整和有神经束结构对鉴别诊断有重要临床意义。结论高频超声诊断鉴别神经鞘瘤的准确度及特异度较高,可为临床医生早期快速诊断提供帮助。     

针吸细胞学在颈部肿块神经鞘瘤诊断中的应用

颈部肿块是临床常见病,其中神经鞘瘤是颈部常见病之一,头颈部占20.6%[1],神经鞘瘤是神经鞘膜细胞发生的良性肿瘤,颈部神经鞘瘤好发于颈侧区,尤以上颈部为多见,手术彻底切除肿瘤是最有效的治疗手段,临床上常需与颈部的其他病变相鉴别,现总结我院术后证实为神经鞘瘤的32例患者术前细针穿刺细胞学诊断资料进行总结分析如下。     

眼部神经鞘瘤的诊断和治疗

目的 探讨眼部神经鞘瘤的临床诊断和病理特征及其治疗。方法 对56例眼部神经鞘瘤住院患者的临床、影像学特点、病理资料和手术情况进行分析。结果 56例均经病理证实为眼部神经鞘瘤,30-50岁多见,占55.4％,多发于肌锥内及眼眶上方,56例手术全切除,其中经前路开眶肿瘤摘除术38例,侧壁开眶肿瘤摘除术14例,眼球摘除术3例,睫状体肿瘤摘除1例,术后随访6个月至12年,2例复发。结论 眼部神经鞘瘤诊断要结合临床及CT、彩超等影像学检查,手术全切除为主要治疗方法。     

恶性外周神经鞘瘤52例临床病理分析

目的探讨恶性外周神经鞘瘤(MPNST)的临床病理情况。方法纳入我院2011-06—2014-06收治的恶性外周神经鞘瘤患者52例为研究对象,收集所有患者临床病理学和免疫学资料,观察恶性外周神经鞘瘤的临床病理特点。结果头颈部和四肢的发生率分别为30.77%、25.00%,明显高于其他部位。所有患者肿瘤细胞S-100蛋白局部病灶阳性,阳性率高达100%。其余表型多呈阴性,阳性率较低。随访2a复发率58.33%,复发患者均在1~12个月内死亡;无瘤生存率33.33%。结论恶性外周神经鞘瘤组织形态复杂,尤其需要与各种肿瘤类型进行鉴别诊断,病死率和复发率极高,预后情况差。     

脊髓髓内神经鞘瘤的临床诊断和治疗

目的 探讨脊髓髓内神经鞘瘤的临床特点和外科治疗方法.方法 回顾分析8例髓内神经鞘瘤的临床特点、影像学表现及其手术治疗效果.结果 8例患者肿瘤均获手术全切除,术后均进行临床随访,随访时间3 - 108个月.术前有肌力下降的5例患者,术后3个月肌力较术前明显改善,有6例患者术后出现不同程度感觉异常:所有患者MRI复查未见肿瘤复发.结论 脊髓背外侧生长且边界清楚,均匀强化的脊髓髓内肿瘤伴有明显神经根症状,应考虑髓内神经鞘瘤诊断,手术全切肿瘤是该病的最佳治疗选择,手术入路和手术技巧与预后密切相关.     

原发性腹膜后神经鞘瘤的诊断与显微外科手术治疗

目的探讨原发性腹膜后神经鞘瘤的诊断和显微外科手术治疗的优缺点。方法对22例原发性腹膜后神经鞘瘤患者的诊断和显微外科手术切除的资料进行回顾性分析。结果 22例患者中肿瘤全切17例(77.3%),次全切5例(22.7%);无严重手术并发症;出血量少,平均每例220 ml。结论原发性腹膜后神经鞘瘤早期诊断困难,术前确切病理诊断依赖肿瘤穿刺活检;显微外科手术切除是一种安全有效的手术方式。     

髓内黑色素性神经鞘瘤

目的报告1例髓内黑色素性神经鞘瘤患者,结合文献探讨其临床和组织病理学特征、诊断与鉴别诊断、治疗原则。方法与结果男性患者,47岁,胸椎椎管内占位性病变切除术后6年、背部疼痛1年。脊椎MRI显示T4~5平面髓内占位性病变。手术全切除肿瘤。组织学形态观察,肿瘤细胞呈梭形或上皮样,排列成片状、巢团状或交织状,胞质内含数量不等的黑色素颗粒,胞核呈圆形或卵圆形,可见小核仁,未见核分裂象。免疫组织化学染色,肿瘤细胞S-100蛋白、黑色素瘤相关抗原HMB45、黑色素-A和Ⅳ型胶原蛋白呈阳性,上皮膜抗原、胶质纤维酸性蛋白、CD57、孕激素受体、结蛋白和肌浆蛋白呈阴性,Ki-67抗原标记指数约为10%。超微结构观察,肿瘤细胞可见连续基膜,胞质内含不同成熟程度的黑色素小体。最终病理诊断为髓内黑色素性神经鞘瘤。随访18个月,肿瘤无复发。结论髓内黑色素性神经鞘瘤临床罕见,其诊断依靠组织学形态、免疫表型和超微结构特征,应注意与黑色素瘤、黑色素细胞瘤和色素性神经纤维瘤相鉴别。髓内黑色素性神经鞘瘤生物学行为较难预测,可局部复发,应对患者进行长期密切随访。     

颈静脉孔区肿瘤的影像分析

目的 探讨颈静脉孔区肿瘤的影像特点。方法 回顾性分析2006年3月至2015年4月经手术病理证实的26例颈静脉孔区肿瘤的CT、MRI和DSA影像资料。结果 神经鞘瘤16例（含恶性1例）,神经纤维瘤2例,颈静脉球瘤2例,脑膜瘤2例,纤维肉瘤、软骨肉瘤、软骨粘液样纤维瘤、软骨样脊索瘤各1例。神经鞘瘤和神经纤维瘤边界清楚多伴有囊变;颈静脉球瘤可见典型的“胡椒盐征”;脑膜瘤可见典型的“脑膜尾征”;纤维肉瘤、软骨肉瘤、软骨黏液样纤维瘤和软骨样脊索瘤常见骨质破坏和瘤内钙化,软骨源性肿瘤T2WI可见特征性高信号。结论 颈静脉孔区肿瘤的影像表现多样,部分征象具有特征性,术前影像学评估能提高临床诊断能力,为外科治疗的选择提供参考。     

Multinodular plexiform tumors of major peripheral nerves: A practical overview

Background and aimsMultinodular/plexiform schwannomas and neurofibromas of major nerves are rare: before surgery, differential diagnosis among these two uncommon variants is challenging. For both forms, surgical removal is recommended in case of progressive growth and worsening of neurological symptoms. Surgery has a higher risk of neurological damage than conventional schwannomas or neurofibromas.In literature, a comparison among these rare tumors is usually limited to the pathological aspect while specific surgical and clinical management indications are lacking. Cutaneous tumors of both forms arising from terminal peripheral nerves’ branches might be treated by plastic surgeons while tumors of major nerves remain under neurosurgical competence. Here we report our recent neurosurgical experience on the matter, to furnish useful suggestions for the management of these tumors.MethodWe analyzed the clinical, radiological, and pathological data in a consecutive case series of plexiform/multinodular nerve tumors operated at our institution in the last five years.ResultsIn our series, neurofibroma type of plexiform tumors was more frequent than schwannoma type: two sporadic plexiform-multinodular schwannomas (patients 1, and 5) and three multinodular/plexiform Neurofibromatosis familial (Neurofibromatosis 1 / NF-1) (patients 2, 3, and 4). Surgery was complex when major nerves were involved. The early outcome appeared mostly related to the pre-surgical neurological conditions and histological grading.InterpretationAlthough sharing some features, multinodular-plexiform schwannomas and neurofibromas have consistent differences from the clinical, surgical and pathological points of view.     

GFA protein reactivity in nerve sheath tumors: a polyvalent and monoclonal antibody study

We studied glial fibrillary acidic (GFA) protein immunoreactivity in 30 schwannomas, including two intracerebral examples, 26 neurofibromas and 12 neuromas using the immunoperoxidase method with a polyvalent antiserum (PVAS) and three well-characterized monoclonal antibody (MAb) preparations. Twelve of the schwannomas, including both intracerebral tumors, two of the neurofibromas and none of the neuromas immunostained with PVAS. Except for one schwannoma, all the PVAS-positive tumors were positive with two of the MAb preparations. While both of the intracerebral schwannomas were positive with the third MAb, none of the extracerebral tumors were. Our results suggest that: 1) human nerve sheath tumors contain cells having polypeptides that share epitopes with GFA protein, but 2) these polypeptides differ from astrocytic GFA protein by at least one epitope, and 3) the location of the tumors in relation to the central nervous system may influence GFA protein immunoreactivity.     

Peripheral and cranial nerve sheath tumors

PURPOSE OF REVIEW: The intention of the authors is to provide the reader with an overview of the recent advances in the diagnosis and treatment of nerve sheath tumors. Vestibular schwannomas, neurogenetic syndromes such as schwannomatosis and multiple isolated neurofibromas, and malignant peripheral nerve sheath tumors are covered in this review. RECENT FINDINGS: Over the last year, literature focusing on different management strategies for patients with vestibular schwannomas dominated the field. Surgical options for this group of patients are changing. Stereotactic radiation is also employed more frequently with promising results. New insights into the biology of peripheral nerve tumor development and growth, including expression of vascular endothelial growth factor by vestibular schwannomas and the role of Notch signaling in malignant transformation of benign neurofibromas have been described. Diagnostic criteria for schwannomatosis, a recently described condition, are being developed. Several cases of multiple isolated neurofibromas and spinal neurofibromas were reported. SUMMARY: Peripheral nerve tumors are classified according to the specific features of cellular differentiation. The most common types include schwannoma and neurofibroma. These tumors can occur sporadically or as manifestations of genetic syndromes such as neurofibromatosis types 1 and 2 or schwannomatosis. The majority of peripheral nerve tumors are benign but malignant transformation does occur. Metastatic tumors can also affect peripheral nerves. The diagnostic modality of choice is magnetic resonance imaging. Positron emission tomography is a useful technique in the presurgical differentiation between benign and malignant peripheral nerve sheath tumors. Treatment is directed towards symptomatic control. Surgery, radiation and, in rare instances, chemotherapy are the major treatment modalities employed.     

Microsurgical management of non-neurofibromatosis spinal schwannoma

IntroductionThe aim of this study is to assess the clinical properties and surgical results of patients diagnosed with spinal schwannomas without neurofibromatosis (NF) properties.Patients and methodsThe data obtained from 35 patients who underwent resection of spinal schwannomas were analyzed. All cases with neurofibromas and those with a known diagnosis of NF Type 1 or 2 were excluded. 35 patients underwent surgery for spinal schwannoma at our institution between January 1997 and 2010. The data were gathered retrospectively from medical records and included clinical presentation, tumor location and post-operative complications. All cases were surgically excised, and they were confirmed to be schwannomas by pathologists with histopathological sections in paraffin stained with hematoxylin–eosin.ResultWe treated 35 (20 males and 15 females) patients with spinal schwannomas. The mean age of the patients was 47.2 (between 13 and 76) years. Of the cases, six schwannomas were located in the cervical spine, four in the thoracic spine, two in cervico-thoracic area, 10 in the thoraco-lumbar area and 13 in the lumbar spine. Two patients had malignant schwannomas that were recurrent. Of the 35 cases, the schwannomas were intradural–extramedullary in 30 cases (86%), intradural–intramedullar in 2 cases (6%), and extradural in 3 cases (9%).ConclusionSpinal schwannomas may occur at any level of the spinal axis and are most frequently intradural–extramedullary. The most common clinical presentation is pain. Most of the spinal schwannomas in non-NF patients can be resected completely without or with minor post-operative deficits. This knowledge may help us to create a strategy for total resection of a spinal schwannomas.     

Differential NF1, p16, and EGFR patterns by interphase cytogenetics (FISH) in malignant peripheral nerve sheath tumor (MPNST) and morphologically similar spindle cell neoplasms

Malignant peripheral nerve sheath tumors (MPNSTs) are diagnostically challenging neoplasms for which sensitive and specific immunohistochemical markers are lacking. Although limited to date, previous studies have suggested that NF1 (17q), NF2 (22q), p16 (9p), and EGFR (7p) alterations may be involved in MPNST tumorigenesis. To determine whether specific genetic changes differentiate between MPNST and morphologically similar neoplasms, we assessed these chromosomal regions in 22 MPNSTs (9 NF1-associated, 13 sporadic), 13 plexiform neurofibromas, 5 cellular schwannomas, 8 synovial sarcomas, 6 fibrosarcomas, and 13 hemangiopericytomas by 2-color FISH. NF1 deletions, often in the form of monosomy 17, were found in MPNSTs (76%). neurofibromas (31%), hemangiopericytomas (17%), and fibrosarcomas (17%), but not in synovial sarcomas or cellular schwannomas. NF1 losses were encountered more frequently in MPNSTs versus other sarcomas (p < 0.001), as were p16 homozygous deletions (45% vs 0%; p < 0.001), EGFR amplifications (26% vs 0%; p = 0.006), and polysomies for either chromosomes 7 (53% vs 12%; p = 0.003) or 22 (50% vs 4%; p < 0.001). Hemizygous or homozygous p16 deletions were detected in 75% of MPNSTs, but not in benign nerve sheath tumors (p < 0.001). Thus, FISH analysis identifies relatively specific genetic patterns that may be useful in selected cases, for which the differential diagnosis includes low- or high-grade MPNST.     

Intracranial intraparenchymal and intraventricular schwannomas: Report of 18 cases

Objective

Intracranial schwannomas of the brain, which are unrelated to the cranial nerves, are extremely rare. In this article, we present a series of eighteen cases of intracranial intraparenchymal and intraventricular schwannomas, which is the largest series to date.

Methods

During the 10-year period from January 2000 to October 2010, we encountered 2491 histologically established cases of intracranial schwannomas, of which only 18 were not related to the cranial nerves. Clinical profiles, radiological features, surgical procedures, intraoperative findings and outcomes were extracted from the patient records and neuroimaging data.

Results

No patients were preoperatively diagnosed with schwannoma. The diagnosis of schwannoma was made by pathological examination with H&E staining and immunohistochemical examination. The 18 cases with intracranial ectopic schwannomas account for 0.8% of all the intracranial schwannomas that were observed within the same time period at our hospital. The age distribution of the patients ranged from 7 to 78 years. There was a slight male predominance: 11 male and 7 female patients (M:F = 1.6:1). Headaches were the most common presenting symptom and were found in most cases. Common neuroradiological characteristics included peritumoral edema and intralesional cysts.

Conclusion

Intracranial intraparenchymal and intraventricular schwannomas are rare, benign neoplasms that cannot be preoperatively differentiated from other parenchymal tumors. Surgical excision is curative, and the long-term prognosis is good. Additional studies are needed to confirm the histogenesis of this schwannoma type.     

Incidentally diagnosed giant invasive sacral schwannoma: Its clinical features and surgical management without stability

Schwannomas are benign encapsulated tumors of Schwan cells that grow slowly along the peripheral myelin nerve fibers. Sacral spinal schwannomas are very rare, and the incidence of sacral schwannoma ranges from 1-5% of all spinal schwannomas, and only around 50 cases are reported in the literature. There are 3 defined types of sacral schwannomas. These are retroperitoneal or presacral, intra osseous, and spinal schwannomas. Patients commonly present with complaints of pain and paresthesia due to the spinal schwannoma extending to extra spinal tissues. Direct x-ray, CT, MRI, and scintigraphy are used for preoperative diagnosis and treatment planning. Local recurrence and transformation to malignancy is very rare. For this reason, the frequently preferred treatments are subtotal removal of the mass or simple enucleation. In our article, we discuss the clinical features and the surgical treatment we performed without the need for stabilization in an incidentally determined giant invasive schwannoma case.Primitive sacral and presacral tumors are very rare. Spinal schwannomas constitute approximately 25% of vertebral tumors. They most commonly tend to settle in the thoracic region. They are very rarely seen in the sacral region and constitute 1-5% of all spinal schwannomas, and only around 50 cases are reported in the literature.1-3 Schwannomas are mostly benign lesions. Malignant ones are either malignant from the beginning or become malignant due to degeneration of benign schwannomas, such as is the case in neurofibromatosis type II patients. There are 3 defined types of sacral schwannomas; retroperitoneal or presacral, intra osseous, or spinal schwannomas. According to the definition by Sridhar et al,2 giant invasive spinal schwannomas are masses that invade more than 2 vertebral levels, invade vertebral bodies, and by extending posteriorly, reach the myofascial regions. For this reason, the clinical and surgical results of giant invasive type spinal schwannomas (GISS) differ slightly from typical schwannomas. The clinical diagnoses of these tumors are made by direct x-ray, CT, and MRI. The ideal treatment of GISS would be to totally remove the tumor by decompression and surgery without creating any neural complications or spinal instability, which would relieve the pressure on neural organs created by the mass and regress symptoms.3-5 Our objective in presenting this particular case is to highlight an interesting and rare presentation for incidentally diagnosed giant invasive schwannoma of the sacrum.     

第四脑室神经鞘瘤的临床特点及手术治疗并文献复习

目的探讨原发性第四脑室神经鞘瘤的临床特征及手术方法。方法分析1例原发性第四脑室神经鞘瘤手术治疗患者的临床资料,并结合文献分析该病的临床特点和手术方法。结果患者为女性,53岁,表现为剧烈头痛、吞咽困难、共济失调、四肢无力等症状,影像学示第四脑室巨大肿瘤,合并脑积水。手术采用经枕下正中小脑延髓裂入路,肿瘤镜下获得全切除,术后病理诊断为神经鞘瘤。术后随访8个月,未见肿瘤复发。结论尽管脑室内神经鞘瘤罕见,且诊断困难,但该类型肿瘤为良性肿瘤,其边界清楚。因此,手术若能获得有效的全切除,远期预后良好。     

Intracranial schwannoma of the hypoglossal nerve

Two patients had a solitary intracranial schwannoma of the hypoglossal nerve. Such schwannomas are rare, and these two cases bring the total number of reported cases to 16. Hemiatrophy of the tongue is always present but is rarely reported by patients. Diagnosis can be facilitated by high-resolution computed tomographic scanning of the posterior fossa and foramen magnum region. The hazards formerly associated with removal of these tumors have largely been eliminated.     

Non-acoustic parastemal schwannomas: results of treatment

The paper presents the results of treatment in 15 cases with non-acoustic parastemal schwannomas, which amounts to 4.3% in this histological subgroup. Among them, there were 7 (2.0%) patients with trigeminal tumors, 5 (1.4%) with facial nerve schwannomas, 3 (0.9%) with schwannomas, which were localized in the jugular foramen. In 4 (57.1%) cases, trigeminal schwannomas were localized in the posterior fossa and in (3.42.9) cases, they spread into the infra- and supratentorial areas. Tumors of the jugular foramen spread extracranially in 2 (66.7%). Their diagnosis was based on clinical intrascopic (computed tomographic and magnetic resonance imaging) data. All the cases had sings of acousticofacial disorders. For tumor removal, the suboccipital retrosygmoid approach was used in 12 cases and combined approaches were applied in 3 cases, including 2 (13.3%) patients in whom schwannomas were removed via two- or three-stage interventions. The anatomic integrity of the cranial nerves adjacent to a tumor was preserved in all the cases. After surgery, their mild or moderate dysfunction that did not lower life quality was observed in most cases. Liquorea and meningitis were seen in 1 (6.7%) case after removal of an extracranial component of trigeminal schwannoma. Radical tumor resection was made in 93.3% of cases. Tumor recurrence was observed in 1 (6.7%) case. There were no fatal cases in a group of the patients operated on. Larger tumors that induce severe deformation of the brain stem should be removed in two steps; this makes it possible to rule out their rapid redislocation that deteriorates the course of an early postoperative period.