peripheral nerve sheath tumors

  • 文章类型: Case Reports
    该病例报告显示了一名22岁女性的T3-T4椎旁神经鞘瘤的异常发生率,由于其非典型表现和生长速度,突出了其临床意义。Schwannmas,良性外周神经鞘瘤,通常生长缓慢,存在轻微或没有神经性症状。然而,这个案子偏离了常规,患者经历了严重的神经性疼痛和在六个月内从37毫米到55毫米的快速肿瘤生长,需要手术干预。这种情况的独特之处在于存在阳性Tinel体征和局部神经性背痛,通常不与椎旁神经鞘瘤相关的特征。通过MRI和组织学评估,神经鞘瘤的诊断得到证实,强调在表现类似症状的患者的鉴别诊断中考虑椎旁神经鞘瘤的必要性。该病例通过强调神经鞘瘤的表现和生长速率的变异性,为医学文献做出了贡献。加强了对出现神经性疼痛和积极神经系统体征的年轻患者进行全面评估和个性化管理的必要性。
    This case report presents an unusual incidence of a T3-T4 paraspinal schwannoma in a 22-year-old female, highlighting its clinical significance due to its atypical presentation and growth rate. Schwannomas, benign peripheral nerve sheath tumors, are typically slow-growing and present with minimal or no neuropathic symptoms. However, this case deviated from the norm, with the patient experiencing significant neuropathic pain and rapid tumor growth from 37 mm to 55 mm over a period of six months, necessitating surgical intervention. Unique to this case was the presence of a positive Tinel sign and localized neuropathic back pain, features not commonly associated with paraspinal schwannomas. Through MRI and histological evaluation, the diagnosis of schwannoma was confirmed, underlining the necessity of considering paraspinal schwannomas in differential diagnoses for patients presenting similar symptoms. This case contributes to the medical literature by emphasizing the variability in presentation and growth rates of schwannomas, reinforcing the need for a thorough evaluation and an individualized approach to management in young patients presenting with neuropathic pain and positive neurological signs.
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  • 文章类型: Journal Article
    本研究报告了一名24岁健康女性中附于下直肌(IR)肌腱的孤立性神经纤维瘤,并回顾了有关临床表现的相关文献。诊断,以及这种罕见肿瘤的治疗。患者接受了成功的肿瘤手术切除,导致相关症状(左下眼睑突出和发红)的解决。病理检查根据特征性组织病理学和免疫组织化学标记证实了神经纤维瘤的诊断。该病例报告强调了孤立性神经纤维瘤以及眼眶和眼附件的原发性肿瘤的罕见性。我们还讨论了源自眼眶和眼附件的孤立性神经纤维瘤的背景。通过手术切除成功处理此病例凸显了准确诊断和量身定制治疗策略的重要性。据我们所知,这是首次报道的仅局限于IR肌腱的孤立性神经纤维瘤.
    The present study reports a case of solitary neurofibroma attached to the Inferior Rectus (IR) muscle tendon in a 24-year-old healthy woman and reviews the relevant literature regarding the clinical presentation, diagnosis, and management of this uncommon tumor. The patient underwent successful surgical resection of the tumor, leading to the resolution of associated symptoms (left lower eyelid protrusion and redness). Pathological examination confirmed the diagnosis of neurofibroma based on characteristic histopathological and immunohistochemical markers. This case report underscores the rarity of solitary neurofibromas and primary neoplasms of orbit and ocular adnexa. We also discuss the background of solitary neurofibromas originating from orbit and ocular adnexa. The successful management of this case through surgical resection highlights the importance of accurate diagnosis and tailored treatment strategies. To the best of our knowledge, this is the first reported solitary neurofibroma confined solely to the IR tendon.
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  • 文章类型: Journal Article
    目的:周围神经鞘瘤(PNSTs)在儿科患者中很少见,尤其是臂丛.缺乏对PNST的研究。本文介绍了一项对PNST诊断和治疗的儿科患者的回顾性队列研究,特别是臂丛神经肿瘤.
    方法:对2007年至2023年在单中心介入治疗臂丛肿瘤的所有儿科患者进行了系统分析。
    结果:研究了11例14例臂丛神经PNST患儿。性别分布为64%的女性和36%的男性,平均年龄10.7岁。91%的人以前有NF-1诊断。右臂丛的患病率较高(64%)。疼痛,Tinel\'ssign,和刚度质量在诊断过程中很常见。43%的患者出现运动障碍。手术是根据症状进行的,特别是疼痛和快速增长,恶性肿瘤风险增加。由于怀疑是恶性肿瘤,进行了具有安全边缘的整块切除术.在患者中,57%获得组织病理学诊断为MPNST(恶性外周神经鞘瘤)。治疗包括放疗和化疗。对所有病例进行临床随访,涉及所有MPNSTs的临床和肿瘤学评估。
    结论:本文介绍了一系列小儿臂丛神经肿瘤,特别是在NF-1中,并强调对该组进行彻底评估的重要性。快速诊断在儿科中至关重要,能够成功手术治疗神经症状有限的小病变,改善长期结果。呼吁可疑群众及时转介专业服务,不管神经症状。良性肿瘤术后进展显示比MPNSTs更好的结果,以完全切除为主要目标。不建议进行针引导活检。
    OBJECTIVE: Peripheral nerve sheath tumors (PNSTs) are rare in pediatric patients, especially in the brachial plexus. Research on PNSTs is lacking. This article presents a retrospective cohort study of pediatric patients diagnosed and treated with PNSTs, specifically brachial plexus tumors.
    METHODS: All pediatric patients intervened in a single center between 2007 and 2023 with brachial plexus tumors were systemically analyzed.
    RESULTS: Eleven pediatric patients with 14 brachial plexus PNSTs were studied. The gender distribution was 64% female and 36% male, with an average age of 10.7 years. Ninety-one percent had a previous NF-1 diagnosis. Right brachial plexus presented a higher prevalence (64%). Pain, Tinel\'s sign, and stiffness masses were common during diagnosis. Motor deficits were noted in 43% of the patients. Surgery was indicated for symptoms, particularly pain and rapid growth, increasing malignancy risk. Due to suspected malignancy, an en bloc resection with safety margins was performed. Among the patients, 57% received a histopathological diagnosis of MPNST (malignant peripheral nerve sheath tumor). Treatment included radiotherapy and chemotherapy. Clinical follow-up was conducted for all cases, involving clinical and oncological evaluations for all MPNSTs.
    CONCLUSIONS: This article present a series of pediatric brachial plexus tumors, especially in NF-1, and emphasizes the importance of thorough evaluation for this group. Swift diagnosis is crucial in pediatrics, enabling successful surgery for small lesions with limited neurological symptoms, improving long-term outcomes. Prompt referral to specialized services is urged for suspected masses, irrespective of neurological symptoms. Benign tumor postsurgical progression shows better outcomes than MPNSTs, with complete resection as the primary goal. Needle-guided biopsy is not recommended.
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  • 文章类型: Journal Article
    背景:周围神经肿瘤是一组罕见的神经外胚层起源的软组织肿瘤。尽管它们中的大多数本质上是良性的,高达10%可能是恶性的。症状取决于部位,尺寸,和被肿瘤压缩的结构。
    目的:为了强调体征和症状的异质性及其表现,这通常使主治医师难以准确诊断并指导患者进行适当的治疗。
    方法:本研究纳入了2015年至2022年在我们三级医院接受治疗的8例患者。对它们进行了详细评估。治疗是手术。患者在放大下进行了完整的肿瘤切除,以帮助保留相邻的神经血管束。术后对所有患者进行随访,以记录其症状状态。
    结果:转诊至我院前的平均持续时间为13个月。七个受试者在演讲中感到疼痛,一个有神经缺陷.七个人也抱怨肿胀。八个病变中有五个是神经鞘瘤,两个神经纤维瘤和一个显示恶性组织学。术后,HoffmanTinel体征在所有六个受试者中都得到了改善。七个受试者中有五个完全没有疼痛,另外两个症状有所减轻。
    结论:在治疗周围神经肿瘤的同时,需要早期诊断和转诊到专科中心才能获得满意的结果。增生性病变应由经验丰富的医生在专科中心进行手术治疗,这些医生具有适当的显微外科手术技能和设备,以确保完全康复。
    BACKGROUND: Peripheral nerve tumors are a group of rare soft tissue tumors of neuro-ectodermal origin. Although the majority of them are benign in nature, up to 10% can be malignant. The symptoms depend on the site, size, and structures compressed by the tumor.
    OBJECTIVE: To highlight the heterogeneity of signs and symptoms and their presentations, which has often made it difficult for the attending physician to accurately diagnose and direct the patient toward appropriate treatment.
    METHODS: Eight patients treated at our tertiary care hospital between 2015 and 2022 were included in this study. They were evaluated in detail. Treatment was surgical. The patients underwent complete excision of the tumor under magnification to help preserve the adjacent neurovascular bundle. All patients were followed up post-operatively to document the status of their symptoms.
    RESULTS: The average duration prior to referral to our hospital was 13 months. Seven subjects had pain at presentation, one had neurological deficit. Seven also complained of swelling. Five of the eight lesions were schwannoma, two neurofibroma and one showed malignant histology. Post-operatively, Hoffman Tinel signs improved in all six subjects. five of the seven subjects were completely pain-free, and the other two had a reduction in symptoms.
    CONCLUSIONS: Early diagnosis and referral to a specialist center are needed to achieve satisfactory outcomes while treating peripheral nerve tumors. Proliferative lesions should be treated surgically in specialist centers by experienced doctors with appropriate skills and equipment for microsurgical procedures to ensure full recovery.
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  • 文章类型: Case Reports
    心肌炎是指由细菌等非传染性病原体引起的坏死的心肌炎症,真菌,或药物。已知念珠菌在健康和免疫受损的个体中引起心肌炎。由于胰岛素分泌受损或功能低下,糖尿病会导致慢性高血糖。诱导一个妥协的状态,并增加感染各种感染的风险。
    我们报告一例自发性糖尿病托里大鼠念珠菌引起的肉芽肿性心肌炎,非肥胖糖尿病模型。
    一只雄性SDT大鼠,61周龄,被安置在传统环境中。向大鼠提供商业饮食和随意的自来水。对心脏进行取样并制备苏木精-和-eosin-的标本,Sirius-red-,Giemsa-,Grocott染色.组织学上,在左心室壁中观察到大肉芽组织的形成。病灶中心显示坏死。此外,病灶周围和心肌细胞之间的炎性细胞浸润和纤维成分增加。偶尔出现在病灶中的Grocott和Giemsa染色阳性细胞团由于其特征性形式而被认为是念珠菌。
    心肌炎的发展和进展可能与糖尿病引起的损害状态有关。
    UNASSIGNED: Myocarditis refers to myocardial inflammation with necrosis caused by non-infectious of infectious agents such as bacteria, fungi, or drugs. Candida is known to cause myocarditis in healthy and immunocompromised individuals. Diabetes mellitus causes chronic hyperglycemia due to impaired secretion or hypofunction of insulin, induces a compromised state, and increases the risk of contracting various infections.
    UNASSIGNED: We report a case of granulomatous myocarditis caused by Candida in a Spontaneously Diabetic Torii rat, a non-obese diabetic model.
    UNASSIGNED: A male SDT rat, 61 weeks of age, was housed in conventional environment. The rat was provided a commercial diet and tap water ad libitum. The heart was sampled and prepared the specimen of hematoxylin-and-eosin-, Sirius-red-, Giemsa-, Grocott-stain. Histologically, formation of large granulation tissue was observed in the left ventricular wall. A center of the foci showed necrosis. Moreover, inflammatory cells infiltration and fibrous component were increased surrounding the foci and between myocardial cells. A Grocott and Giemsa staining-positive cell masses occasionally appearing in the foci were considered to be Candida because of their characteristic form.
    UNASSIGNED: The development and progression of myocarditis were potentially related to a diabetes-induced compromised state.
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  • 文章类型: Case Reports
    恶性triton肿瘤(MTT)是恶性周围神经鞘瘤(MPNST)的亚型,由周围神经或神经纤维瘤的Schwan细胞发展而来,并显示横纹肌母细胞分化。是一种罕见的软组织肿瘤,预后较差。
    我们报告了一名46岁男性患者的右肩恶性Triton肿瘤(MTT)病例,该患者于2018年6月在侯赛因国王医疗中心皇家康复中心的肌肉骨骼肿瘤诊所就诊。
    患者主诉8个月的进行性右肩疼痛和肩后外侧区肿胀。在这种情况下,准确的诊断至关重要,包括X线和磁共振成像(MRI)在内的研究表明,软组织肿瘤累及右肩区域,从而对侵袭性软组织肿瘤进行了鉴别诊断,并制定了开放切开活检的计划,以组织病理学报告为一例。恶性Triton肿瘤是一种非常罕见且侵袭性的肉瘤,起源于周围神经鞘,因为它是恶性周围神经鞘肿瘤的亚型,此后对整个肿瘤进行了辅助切除,并进行了安全化疗。
    选择的治疗方法是广泛的肿瘤切除,然后进行化疗和/或放疗,以提高5年生存率。
    UNASSIGNED: Malignant triton tumors (MTT) are subtype of malignant peripheral nerve sheath tumor (MPNST) which develop from Schwan cells of peripheral nerves or within neurofibromas, and shows rhabdomyoblastic differentiation. It is a rare soft tissue tumor with poor prognosis.
    UNASSIGNED: We report a case of Malignant Triton Tumor (MTT) arising in the right shoulder in a 46 year old male patient presented to our Musculoskeletal Oncology Clinic at Royal Rehabilitation center at King Hussein Medical Center during June 2018.
    UNASSIGNED: The patient was complaining of an 8 months long progressive right shoulder pain and swelling at the posterior lateral area of the shoulder. As accurate diagnosis is crucial in such case, investigations that included x-rays and magnetic resonance imaging (MRI) demonstrated an soft tissue tumor involving the right shoulder area leading to the differential diagnosis of aggressive soft tissue tumor which laid down the plan of an open incisional biopsy to be reported histopathological as a case of Malignant Triton Tumor which is a very rare and aggressive sarcoma originates from the peripheral nerve sheaths as it is subtype of malignant peripheral nerve sheath tumors after which excision of the entire tumor with safety margin was performed and referred for adjuvant chemotherapy.
    UNASSIGNED: The treatment of choice is radical tumor excision with wide margins followed by chemotherapy and /or radiotherapy to improve the 5 years survival rates.
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  • 文章类型: Journal Article
    周围神经鞘瘤(PNST)包括神经鞘瘤和神经纤维瘤。在磁共振成像检查中,良性PNST周围脂肪组织增加的发现被描述为“分裂脂肪标志”。提示肿瘤的肌内或肌间位置。然而,很少有研究将此体征描述为PNST的显着超声特征。这项研究的主要目的是回顾性评估高分辨率超声诊断的良性PNST周围脂肪组织沉积的增加。此外,我们的目的是证实受影响区域周围血管形成的存在。回顾性分析10例PNST和2例高分辨率超声诊断的创伤后神经瘤,采用Logiq®P8超声,采用2-11MHz多频线性探头L3-12-D(中心频率:10MHz)。任何位置的局部类型的神经纤维瘤和神经鞘瘤均被视为椭圆形或梭形的低回声肿瘤。在6例中观察到了出入神经(尾巴征)。显示在肌间和皮下位置的局部病变。在孤立的肌肉间和肌肉内周围神经鞘瘤的情况下,注意到高回声组织(脂肪分裂征)的存在。主要是神经鞘瘤.虽然小肿瘤没有显示出尾巴的迹象,在US上脂肪组织和血管的增加得到了很好的证明.总之,神经的连续性构成了PNST超声诊断的基础。然而,高分辨率US可以令人信服地证明上极和下极以及肌肉间或肌肉内良性PNST中脂肪的存在增加。
    Peripheral nerve sheath tumors (PNST) comprise schwannomas and neurofibromas. The finding of increased adipose tissue around benign PNSTs has been described as the \"split fat sign\" on magnetic resonance imaging exams, which is suggestive of an intramuscular or intermuscular location of the tumor. However, few studies have described this sign as a salient ultrasound feature of PNSTs. The main purpose of this study was to retrospectively evaluate the presence of increased fatty tissue deposition around benign PNSTs diagnosed by high-resolution ultrasound. In addition, we aimed to corroborate the presence of vascularization around the affected area. A retrospective analysis of ten cases of PNSTs and two cases of post-traumatic neuromas diagnosed by high-resolution ultrasound was performed with a Logiq® P8 ultrasound with a 2-11 MHz multifrequency linear probe L3-12-D (central frequency: 10 MHz). Localized types of neurofibromas and schwannomas in any location were seen as predominantly hypoechoic tumors with an oval or fusiform shape. Exiting and entering nerves (tail sign) were observed in six cases, showing localized lesions both in intermuscular and subcutaneous locations. The presence of increased hyperechoic tissue (the split fat sign) was noted in cases of solitary intermuscular and intramuscular peripheral nerve sheath tumors, mainly the schwannomas. Though small tumors did not demonstrate the tail sign, the increase in adipose tissue and vascularity on US was well demonstrated. In conclusion, the nerve in continuity forms the basis of the ultrasonographic diagnosis of PNSTs. However, high-resolution US can convincingly demonstrate the increased presence of fat in the upper and lower poles as well as circumferentially in intermuscular or intramuscular benign PNSTs.
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  • 文章类型: Journal Article
    目的:在1型神经纤维瘤病(NF1)患者的脑PET和F-18氟脱氧葡萄糖(FDG)中,丘脑代谢减退是一个一致的发现。然而,这种代谢改变的病理生理学是未知的。我们假设这可能是NF1特征性外周神经鞘瘤(PNST)对丘脑外周输入的干扰所致。为了检验这个假设,我们调查了丘脑FDG摄取与数量之间的关系,volume,和PNST的本地化。
    方法:这项回顾性研究包括22名成年NF1患者(41%的女性,36.2±13.0年)指的是全身FDG-PET/对比增强CT,用于怀疑PNST的恶性转化以及22个性别和年龄匹配的对照。大脑FDG摄取按体素缩放至小脑灰质的个体中位摄取。使用左右对称的解剖丘脑面罩确定丘脑FDG摄取的双侧平均和左右不对称性。在对比增强CT中手动分割PNST。
    结果:与对照组相比,NF1患者的丘脑FDG摄取减少了2.0个标准差(p<0.0005)。左右不对称性增加了1.3个标准差(p=.013)。≥3个PNSTs的NF1患者的丘脑低代谢高于≤2个PNSTs的患者(2.6vs.1.6标准偏差,p=.032)。椎旁/椎旁PNST的发生和平均PNST体积对丘脑FDG摄取的影响未达到统计学意义(p=.098和p=.189)。丘脑FDG摄取的左右不对称性与PNST负荷的左右不对称性无关(p=.658)。
    结论:这项研究提供了NF1中丘脑低代谢的左右不对称性的初步证据,并且可能由NF1相关的外周肿瘤介导。
    Thalamic hypometabolism is a consistent finding in brain PET with F-18 fluorodeoxyglucose (FDG) in patients with neurofibromatosis type 1 (NF1). However, the pathophysiology of this metabolic alteration is unknown. We hypothesized that it might be secondary to disturbance of peripheral input to the thalamus by NF1-characteristic peripheral nerve sheath tumors (PNSTs). To test this hypothesis, we investigated the relationship between thalamic FDG uptake and the number, volume, and localization of PNSTs.
    This retrospective study included 22 adult NF1 patients (41% women, 36.2 ± 13.0 years) referred to whole-body FDG-PET/contrast-enhanced CT for suspected malignant transformation of PNSTs and 22 sex- and age-matched controls. Brain FDG uptake was scaled voxelwise to the individual median uptake in cerebellar gray matter. Bilateral mean and left-right asymmetry of thalamic FDG uptake were determined using a left-right symmetric anatomical thalamus mask. PNSTs were manually segmented in contrast-enhanced CT.
    Thalamic FDG uptake was reduced in NF1 patients by 2.0 standard deviations (p < .0005) compared to controls. Left-right asymmetry was increased by 1.3 standard deviations (p = .013). Thalamic hypometabolism was higher in NF1 patients with ≥3 PNSTs than in patients with ≤2 PNSTs (2.6 vs. 1.6 standard deviations, p = .032). The impact of the occurrence of paraspinal/paravertebral PNSTs and of the mean PNST volume on thalamic FDG uptake did not reach statistical significance (p = .098 and p = .189). Left-right asymmetry of thalamic FDG uptake was not associated with left-right asymmetry of PNST burden (p = .658).
    This study provides first evidence of left-right asymmetry of thalamic hypometabolism in NF1 and that it might be mediated by NF1-associated peripheral tumors.
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  • 文章类型: Journal Article
    颈部混合神经鞘瘤/神经纤维瘤并不常见,分类为周围神经鞘瘤(PNSTs)。PNST从软组织发展而来,其中神经鞘瘤,神经鞘瘤,和神经纤维瘤是最常见的。混合PNST由1种以上的PNST组成,如混合神经鞘瘤/神经纤维瘤。由于对该主题的研究有限,这些肿瘤的确切流行病学和发病机理仍在很大程度上未知。这种肿瘤可以扩散到软组织上,尽管大多数病例报道涉及皮下层或真皮。一些研究表明,混合神经鞘瘤/神经纤维瘤可能与神经纤维瘤病有关。我们介绍了一例51岁的女性患者,通过组织病理学和免疫组织化学诊断为颈部混合神经鞘瘤/神经纤维瘤。患者被转诊到神经内科进行神经纤维瘤病筛查,报告阴性结果。12个月后,患者没有肿瘤复发的证据.
    Neck hybrid schwannoma/neurofibromas are uncommon and classified as peripheral nerve sheath tumors (PNSTs). PNSTs develop from soft tissues, of which schwannoma, perineurioma, and neurofibroma are the most common. Hybrid PNSTs consist of more than 1 type of PNST, such as hybrid schwannoma/neurofibromas. The exact epidemiology and pathogenesis of these tumors are still largely unknown because of the limited studies on this topic. Such tumors can spread over the soft tissues, although most cases reported involve the subcutaneous layer or dermis. Some studies have suggested that hybrid schwannoma/neurofibromas may be associated with neurofibromatosis. We present a case of a 51-year-old female patient with a neck hybrid schwannoma/neurofibroma diagnosed by histopathology and immunohistochemistry. The patient was referred to the neurology department for neurofibromatosis screening, which reported negative results. After 12 months, the patient showed no evidence of tumor recurrence.
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  • 文章类型: Case Reports
    神经纤维瘤,良性外周神经鞘瘤,代表一个罕见的原因后骨间神经综合征。电诊断研究可能无法确定神经压迫的确切部位,可能的损伤,压迫神经,不提供有关形态变化的信息。超声波是一种具有成本效益的,提供周围神经动态跟踪机会的实用模式,它被广泛认为是周围神经的初始成像模式。在这里,我们报道一例13岁男孩骨间后神经麻痹,经超声诊断为骨间后神经神经纤维瘤。本病例报告强调了超声在定位和确定骨间后神经麻痹病因方面的益处。对于可疑的周围神经病变,建议进行细致的超声检查,无论电生理和成像方式的结果如何。
    Neurofibroma, a benign peripheral nerve sheath tumor, represents a rare cause of posterior interosseous nerve syndrome. Electrodiagnostic studies may not identify the exact site of nerve compression, a possible lesion that compresses the nerve and do not provide information about the morphological changes. Ultrasound is a cost-effective, practical modality that provides the opportunity for dynamic tracking in the peripheral nerves, and it is widely considered as the initial imaging modality for peripheral nerves. Herein, we report a case of posterior interosseous nerve palsy in a 13-year-old boy with neurofibroma of posterior interosseous nerve diagnosed with ultrasound. The benefit of ultrasound in localizing and determining the etiology of the posterior interosseous nerve palsy is emphasized in this case report. A meticulous ultrasound examination is recommended in suspected peripheral nerve lesions, regardless of the results of electrophysiological and imaging modalities.
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