神经纤维瘤病MR影像表现

目的　分析并探讨神经纤维瘤病的分型及各型的MR影像学表现。方法　回顾性地分析 8例神经纤维瘤病患者的临床及影像学资料 ,所有病例均常规行MRI及钆剂强化扫描 ,归纳所有患者的临床表现及影像学特点。结果　根据NIH的诊断标准 ,8例中 ,4例属NF -1型 ,均在脑内胶质瘤、椎管内神经纤维瘤或脑内对称部位的异常信号基础上 ,合并有皮肤的咖啡牛乳色斑或多发神经纤维瘤 ,其中 1例有家族史 ;4例属NF -2型 ,均患双侧听神经瘤 ,2例合并髓内星型细胞瘤。结论　NF -1型在所有神经纤维瘤病中占大多数 ,且多合并有皮肤损害 ;NF -2型较少见 ,典型表现为双侧听神经瘤 ,皮损少见。MRI及强化扫描对检出神经纤维瘤病中枢神经系统的损害方面有优势     

神经纤维瘤病的CT和MRI表现

目的:探讨神经纤维瘤病的CT和MRI影像表现,并就二者的优劣进行比较.方法:分析17例神经纤维瘤病的临床特点和CT、MRI表现,并就NFⅠ型与其它神经皮肤综合症、NFⅡ型与常见发生于桥小脑角区的肿瘤进行了鉴别.结果:17例神经纤维瘤病中,NFⅠ型6例,NFⅡ型11例.CT显示NF不及MRI,后者能够全面准确地显示病灶分布,病变大小、形态和信号特征以及与相邻组织结构的关系,特别是MR增强扫描能够显现伴发的颅内小病灶.结论:MRI是目前诊断神经纤维瘤病的最佳影像学检查方法,优于CT.     

神经纤维瘤病的影像诊断10例分析

病例资料　回顾性分析 10例神经纤维瘤病的影像学表现 ,其中男 4例 ,女 6例 ,年龄 13～ 5 3岁 ,5例有家族史。NF Ⅰ型 8例 ,同时具有Ⅰ型和Ⅱ型特点的 2例。临床上除 2例分别有听力和视力障碍 (听神经瘤、视神经肿瘤 )外 ,均无明显症状。全部病例符合神经纤维瘤病的临床诊断标准。影像学检查包括X线平片、CT平扫、MRI( 7例行增强扫描 )。10例中 ,6例均有皮肤或皮下的多发性神经纤维瘤 ,另外伴随的局部异常有 :右侧听神经瘤 ;蝶骨大翼缺损、局部蛛网膜囊肿及相邻脑叶的异常 ;T1 、T2 椎弓缺损并脊膜膨出 ;椎旁肿物并寰枢椎脱位和C2 4椎…     

中枢型神经纤维瘤病的临床表现与MR影像特征(附4例报告及文献复习)

目的 研究中枢型神经纤维瘤病（神经纤维瘤病2型，NF2）的临床表现与MR影像特征。方法 4例可疑NF2患于临床症状出现或加重后3周至2年内行头颅Gd-DTPA增强前后MRI检查，NF2的MRI诊断得到手术与病理证实。同时，记录了每例患的临床表现。结果 MRI平扫显示，NF2病灶的特征性表现为T1WI上呈低，等信号，而在T2WI上呈高信号，Gd-DTPA增强后，病灶呈明显强化，MRI还证实了4例NF2患均合并脑膜瘤，这些患最常见的症状是进行性耳聋，耳鸣，眩晕，步态不稳。结论 NF2具有恒常的能通过对比增强MR像证实的强化特征，因此，头颅Gd-DTPA增强前后MRI检查应成为诊断MF2的主要手段。     

神经纤维瘤病Ⅰ型的MRI影像学表现

目的分析神经纤维瘤病I型(NF1)的MRI影像学表现,提高对本病的认识。方法回顾性分析26例确诊神经纤维瘤病I型患者的临床及影像学资料,26例患者均接受MRI的检查,3例患者同时接受了CT的检查,1例患者同时接受了X线检查。结果 26例神经纤维瘤病I型(NF1)中有16例颅内中枢神经系统肿瘤,5例椎管内肿瘤,1例腹腔及腹膜后内肿瘤,1例臀部丛状神经纤维瘤,1例膝关节及2例小腿肿瘤,20例合并有皮肤的咖啡牛乳色斑,12例有家族史。结论神经纤维瘤病I型具有典型MRI表现,与临床表现结合可以确诊该病。     

神经纤维瘤病I型脑内影像学表现探讨

目的探讨神经纤维瘤病I型(NF1)患者脑内影像学表现。方法回顾性分析16例临床确诊NF1患者头颅影像学资料,患者均行常规MRI平扫(T_2 WI、T_1 WI、FLAIR),增强扫描。结果 11例病变表现为基底节受累的T_2/FLAIR高信号,其中1例单侧,10例双侧;累及丘脑5例,脑干受累4例;脑内病灶形态呈斑片状,T_2WI序列共检出27个,FLAIR序列共检出39个。仅1例患者病灶呈肿块样改变;5例增强未见强化;1例MRS显示Cho/Cr:1.93,Cho/NAA:2.52;DWI未见弥散受限。其中6例CT平扫显示阴性。结论 NF1患者在颅脑具有特征性MRI表现,基底节好发,FLAIR序列较T_2序列检出病灶更具有优势;磁共振在NF1患者基底节病变的检出及定性、随诊方面具有明显优势。     

神经纤维瘤病I型的MRI研究

目的：回顾神经纤维瘤病I型（NF1）患者MRI表现，分析MR扫描序列及其诊断价值，以建立合适的MR成像方案，为NF1影像诊断提供有价值的依据。方法：对30例临床确诊为NF1患者采用本组MR成像方案进行扫描，主要包括：轴面SE序列T2WI；平扫矢状面SE脉冲序列T1WI；增强轴面或矢状面SE脉冲序列T1WI；轴面或冠状面液体衰减反转恢复（FLAIR）序列，同时分析病变的发病部位、数目、形态、信号的变化和病变的强化情况等。结果：MRI可见下列3种表现：（1）多发性脑内错构瘤：30例中25例在SE脉冲序列T2WI和FlAIR脉冲序列见高信号病灶，病灶主要位于苍白球、小脑和脑干。另外，25例中20例可见海马回、海马旁回等区晕状高信号改变。（2）视通道或下丘脑胶质瘤：视神经、视交叉增粗、扭曲；视交叉或下丘脑肿块，SE脉冲序列T2WI和FlAIR序列表现为不规则分叶状混杂信号肿块，在增强SE脉冲序列T1WI有明显不规则强化。（3）脊柱多发性神经纤维瘤：SE脉冲序列T2WI和脂肪抑制短时反转恢复（STIR）序列显示高信号沿脊神经分布的多发性肿瘤。结论：MRI能够作为1种 常规的影像检查方法对NF1患者进行诊断和追踪。本组MR成像方案能较好地显示NF1的多发性或多灶性病变。     

中枢神经系统黑色素细胞肿瘤的影像学分析

目的 分析总结中枢神经系统黑色素细胞肿瘤的CT和MRI表现.资料与方法 回顾性分析15例经手术病理证实的中枢神经系统黑色素细胞肿瘤患者的CT、MRI影像特点.男9例,女6例,平均年龄33岁.全部病例行MR平扫及增强扫描,5例颅内病例行CT平扫.结果 颅内黑色素细胞肿瘤10例,其中单发7例,多发3例;椎管内5例,单发4例,多发1例,共6个病灶.幕上6例,幕下3例,骑跨幕上下1例.椎管内2个病灶位于颈段,2个病灶位于胸段,2个病灶位于胸腰段.颅内病灶位于脑外或邻近脑表面,椎管内1例位于髓内,其余位于髓外硬膜下.CT平扫2例为低密度,3例为高密度.MR平扫,9例为显著短T1、短T2信号,其他信号表现各异.增强扫描均为明显强化.4例脑膜广泛转移.结论 中枢神经系统黑色素细胞肿瘤具有典型的影像表现,尤其是MRI信号特点具有特征性,术前可以做出准确诊断.     

椎管内脊膜瘤及神经源性肿瘤MRI增强影像的特征性分析

目的:分析椎管内脊膜瘤及神经源性肿瘤MR增强影像的特征。方法:搜集经手术病理证实的椎管内脊膜瘤11 例,神经源性肿瘤12例,全部病例均行MR平扫及增强扫描。结果:椎管内脊膜瘤MR增强影像特征为“肿瘤周边重度环状增强”、脊膜“尾巴征”;神经鞘瘤MR增强影像特征为多灶样不增强;神经纤维瘤MR增强影像特征为病灶内星芒状不增强。结论:椎管内脊膜瘤及神经源性肿瘤都有各自的MR增强影像特征,其对定性诊断有重要价值     

节细胞神经瘤的CT及MRI表现

【摘要】目的：探讨节细胞神经瘤的CT及MRI表现，旨在提高对该病的影像诊断水平。方法：搜集43例经手术病理证实的节细胞神经瘤的临床及影像资料，患者术前行CT或MRI平扫及多期增强扫描，结合文献回顾性分析其影像特征。结果：后纵隔脊柱旁19例（右侧14例，左侧5例）；肾上腺10例（右侧7例，左侧3例）；椎管内外多发6例，其中3例合并Ⅰ型神经纤维瘤病；1例合并Ⅱ型神经纤维瘤病；椎管内外单发2例；腹膜后5例。43例均边界清楚，25例呈椭圆形或梭形，9例呈类圆形，9例形态不规则。22例伴有伪足或呈嵌入式生长，13例脊柱旁病灶部分嵌入椎管内，造成相应椎间孔扩大。CT平扫9例出现钙化，30例CT增强扫描，其中22例动脉期及静脉期无明显强化，5例轻度不均匀强化，3例中度强化，9例延迟期可见条片状或絮状强化。MR T1WI呈等或稍低信号，T2WI以高信号为主，其中8例可见条索状稍低信号。12例MR增强扫描，其中8例呈轻度斑片状或絮状不均匀强化，1例呈明显不均匀强化。结论：节细胞神经瘤的影像表现具有一定的特征性，若病灶位于后纵隔、腹膜后或肾上腺，边界清楚，形态规则，呈伪足或嵌入式生长，密度/信号均匀，CT可见钙化或MR见漩涡征，增强扫描条索状或絮状轻度强化，应首先考虑诊断节细胞神经瘤。     

腮腺腺淋巴瘤的CT和MRI表现

目的探讨腮腺腺淋巴瘤(Warthin瘤)的CT及MRI表现,旨在提高对本病的认识。资料与方法回顾性分析经手术病理证实的14例腮腺淋巴瘤的CT和MRI表现。12例行CT平扫,其中10例行增强扫描;2例行MRI平扫,其中1例行增强扫描。结果 14例共22个病灶,单侧多发3例,单侧单发9例,双侧单发2例且其中1例为术后14年对侧再发。16个(72.7%)病灶或病灶主体位于腮腺浅叶后下极,长径0.8～5.0cm,平均长径(2.3±1.3)cm,病灶呈圆形或卵圆形;19个(86.4%)病灶边缘光整,3例单发病灶边缘模糊,手术证实合并感染。12例(共18个病灶)CT平扫14个(77.8%)病灶密度均匀,4个(22.2%)病灶密度不均匀;10例(共15个病灶)增强扫描7个(46.7%)病灶呈明显强化,5个(33.3%)病灶呈中度强化,3个(20%)病灶呈轻度强化。3个病灶可见边缘线样强化。MRI上肿瘤呈均匀或不均匀信号,T1WI呈低信号,T2WI呈中等或高信号;平扫包膜呈环状低信号,增强扫描呈轻度强化。结论中老年男性,病灶位于腮腺后下极,边缘清晰,明显强化,特别是双侧或多发病灶,并有吸烟史者,应首先考虑腮腺腺淋巴瘤的诊断。     

中枢神经系统黑色素瘤的影像诊断

目的：分析中枢神经系统黑色素瘤的MRI影像特征。材料和方法：10例中枢神经系统黑色素瘤患者，其中男女各5例，年龄11－59岁。9例病灶位于颅内，其中3例有皮肤黑色素瘤切除史；1例位地颈髓。10例全部行MRI检查，其中4例增强MRI扫描，2例CT平扫。结果：9例MRI均表现为不均－短T1、短T2信号，并在T2WI可见低信号肿块影，另1例显示不均一长T1短T2信号。4例为不均一明显强化，其中1例沿软脑膜转换；2例CT平扫为血肿密度。结论：MRI显示黑色素瘤具有特征性，显示脑膜浸润的范围和程度优于CT。     

Encephalic lesions in neurofibromatosis: clinical manifestations and typical magnetic resonance findings

Neurofibromatosis is the commonest neuroectodermal disease. It is characterized by dysplasias and/or tumors of organs and tissues derived from the embryonic ectoderm, mesoderm and endoderm, and most frequently presents with nervous system and cutaneous lesions. It can be classified as neurofibromatosis type 1 (NF-1 or von Recklinghausen disease) and neurofibromatosis type 2 (NF-2 or bilateral acoustic neurofibromatosis). In order to assess clinical presentation of the disease and diagnostic value of Magnetic Resonance Imaging (MRI), the authors retrospectively evaluated the clinical records and the cranial MR studies of 21 patients with neurofibromatosis (18 with NF-1 and 3 with NF-2). Distinctive abnormalities between the two types were found in both clinical presentation and MR studies. Clinically, NF-1 patients presented most often with blindness, while NF-2 patients were deaf and had fewer cutaneous lesions. The evaluation of MR studies showed that NF-1 patients were more likely to be affected with intracranial gliomas, predominantly of the optic pathways. Moreover, foci of prolonged T2 relaxation were frequently observed, primarily in the globus pallidus of the basal ganglia and in the dentate nucleus of the cerebellum. Some of the foci in the globi pallidi exhibited increased signal intensity on T1-weighted images as well. NF-2 patients more frequently presented with bilateral acoustic schwannomas, meningiomas and cerebral white matter foci of prolonged T2 relaxation, but they did not have dentate and basal ganglia lesions. The authors conclude that as a rule the manifestations of NF-1 and NF-2 on cranial MRI are separate and distinct; they do not overlap. MRI is an useful clinical tool for the diagnosis and the follow-up of patients with neurofibromatosis.     

Extracerebral neoplastic manifestations in neurofibromatosis 1: integrated diagnostic imaging

PURPOSE: To analyze the extracerebral manifestations of type 1 neurofibromatosis (NF-1), with special reference to peripheral nerve tumors. MATERIAL AND METHODS: The findings of 376 NF-1 patients (194 men and 182 women; age range: 0.1-48 years, mean: 8.1) were retrospectively reviewed. The patients had been submitted to abdominopelvic and superficial US and, in case of abnormal US findings or in the presence of symptoms, to CT and/or MRI. In addition, we considered 5 more patients (2 men and 3 women; age range: 50-72 years, mean: 64.4) with incomplete forms of NF-1 diagnosed after the finding of nerve sheath tumors. Biopsy (12 cases), surgery (10 cases), or clinical-instrumental follow-up were the study criteria. RESULTS: In the first group of patients we identified 91 cutaneous, 222 subcutaneous, 11 pendulous and 25 internal neurofibromas. Plexiform neurofibromas were found in the neck (1 case), chest (6 cases), abdomen (16), pelvis (8). We also found 1 benign and 1 malignant Schwannomas, 2 nerve sheath fibrosarcomas, 1 dopamine-producing sympatoma and 1 spermacytoma. As for the second group of patients, we had 2 Schwannomas, 1 pulmonary neurofibroma, and 2 multiple plexiform neurofibromas. The neurofibromas exhibited homogeneous US hypoechogenicity or slight echogenicity, with little contrast enhancement at CT. MR showed peripheral hyperintensity and central hypointensity on T2-weighted sequences and marked contrast enhancement after gadolinium, sometimes with mostly central uptake. The plexiform neurofibromas, which are typical of NF-1, had poorly-defined or infiltrating margins, with similar findings to the previous ones but sometimes with less homogeneous patterns at both US and CT. The Schwannomas, which are a less common finding in NF-1, exhibited different features at MRI and CT, namely pseudo-liquid or solid-inhomogeneous patterns with irregular and inhomogeneous contrast enhancement relative to the Antoni A/B tumor component. In the malignant lesions we observed infiltrating patterns, with irregular and inhomogeneous contrast enhancement, arranged asymmetrically relative to the contralateral lesion. CONCLUSIONS: Extra-axial neoplasms are a frequent finding in NF-1. Despite the extremely variable appearances of some lesions (particularly Schwannomas), the typical plexiform neurofibroma exhibits characteristic patterns. The diagnosis of malignancy often requires bioptic confirmation.     

臂丛区神经源性肿瘤的X线平片、CT及MRI表现（附5例报告）

报道５例臂丛神经源性肿瘤，主要描述其影像学表现。５例中，男性４例，女性１例，年龄３２岁～６６岁，临床症状无特殊。影像学检查方法包括胸部正、侧位片（ｎ＝４），颈椎平片（ｎ＝３），ＣＴ扫描（ｎ＝４）及ＭＲＩ检查（ｎ＝４）。病变位于右侧者４例，左侧１例，均经手术及病理证实，其中神经鞘瘤３例，神经纤维瘤２例。普通Ｘ线表现包括肺尖区肿块（ｎ＝３），椎间孔扩大（ｎ＝１）。ＣＴ所见：肿块呈梭形（ｎ＝２）或哑铃状（ｎ＝２），平扫密度与肌肉ＣＴ值相近，注射造影剂后肿块增强幅度高于肌肉。ＭＲＩ表现：Ｔ１加权像上肿瘤信号与肌肉相近３例，略低于肌肉信号１例；Ｔ２加权像显示病变均为高信号。初步结论：根据病变的分布及上述影像学表现，臂丛神经源性肿瘤可于手术前做出诊断。     

MRI动态增强扫描在肝脏炎性假瘤诊断与鉴别中的价值

目的探讨MRI平扫及动态增强在肝脏炎性假瘤(IPL)诊断与鉴别中的价值。资料与方法回顾性分析行Gd-DTPA动态增强MRI检查并经手术病理证实的IPL12例13个病灶,重点观察病灶在动态增强扫描中的强化方式及特点。结果12例患者共发现13个病灶,在T1WI上,4个病灶呈等信号,其余呈低信号。在T2WI上,6个病灶呈高信号,4个呈等信号,3个呈低信号。动态增强扫描后,病灶强化方式及特点如下:(1)全部病灶呈延迟强化;(2)动脉期4个病灶可见强化,其余无明显强化;(3)门静脉期和延迟期可见周边部环形强化(9/13,69.23%)、分隔样强化(6/13,46.15%)、中心或偏心性小结节状强化(2/13,15.38%)、全瘤不均质强化(1/13,7.69%);(4)延迟期病灶内存在无强化区(7/13,53.85%);(5)延迟期病灶缩小感(4/13,30.77%)。结论MRI动态增强扫描可反映IPL的血供特点及病理特征,对定性诊断与鉴别有较高的临床价值。     

Central nervous system lesions in neurofibromatosis. Clinical, MRI and histopathological correlations. A trial of classification]

The National Institute of Health Consensus Panel on Neurofibromatosis (NF) recently recognized 2 distinct forms of NF (NF-1 and NF-2) and stated that variant forms may exist. We selected 30 patients who fulfilled the criteria of NF-1 or whose condition was consistent with NF-2. All patients showed pathological magnetic resonance images (MRI), and in 19 cases confirmation was obtained from histopathology. We established correlations between the site and nature of the lesions on the one hand and the diagnostic criteria of NF on the other hand, there by hoping to contribute to a better knowledge and classification of neurofibromatosis. Nineteen patients had only intraparenchymatous lesions of the central nervous system (CNS) and fulfilled the criteria of NF-1; histopathological examination demonstrated pilocytic astrocytoma in 8 cases. Eleven patients showed only extra-axial lesions; 8 of them had criteria suggestive of NF-2, except for familial history. Pathological examination revealed either acoustic, pluriradicular of mixed schwannomas (7/8) or pluriradicular ganglioneuromas (1/8). Two patients had unilateral extra-axial pluriradicular cervical lesions and fulfilled the diagnostic criteria of NF-1; pathological examination revealed neurofibroma in both cases. One female patient had both intra- and extra-axial lesions that fulfilled the criteria of NF-1 and NF-2, suggesting the existence of a mixed form (NF-3).     

不典型神经鞘瘤的影像学诊断

目的 探讨不典型神经鞘瘤的临床影像学表现特点。方法 回顾分析44例经病理学诊断的不典型神经鞘瘤的CT和MRI表现。结果 CT表现为类圆形或分叶状低密度或等低混杂密度灶,绝大多数（38/40）边界清楚,其中实性肿瘤17例,囊实性病灶19例,单纯囊性病灶4例。CT增强扫描肿瘤实质呈渐进性不均匀强化。囊性病灶内存在强化程度不一的结节灶可能是囊实性神经鞘瘤较为特征性的表现。实性病灶呈＂同心圆样＂强化可能是实性神经鞘瘤比较有价值的征象。MRI上肿瘤实质部分呈稍长T1稍长T2信号,囊性部分呈长T1长T2信号,病灶周围可见水肿信号;＂靶征＂是特征性的MRI表现。结论 不典型神经鞘瘤可发生在身体任何部位,熟悉其影像学特点对诊断很有帮助。     

颅骨嗜酸性肉芽肿的CT和MRI诊断

目的探讨颅骨嗜酸性肉芽肿的CT和MRI表现，提高对其诊断和鉴别诊断水平。资料与方法回顾性分析21例经手术病理证实的颅骨嗜酸性肉芽肿的CT和MRI表现，17例行CT平扫，12例行MR检查，10例行MR增强扫描。结果21例共发现24个病灶，其中单发19例（眼眶2例，斜坡2例，额骨9例，颞骨1例，顶骨4例，枕骨1例），多发2例。CT像上主要表现为颅骨板障的破坏，边界清楚，部分病灶内可见残留的小骨片以及大小不等的软组织肿块，密度不均匀。MR表现为T1低信号，T2高信号，CT和MRI增强扫描明显强化。结论颅骨嗜酸性肉芽肿的CT和MRI表现有一定的特征，CT和MRI有助于明确颅骨嗜酸性肉芽肿的诊断。