BPNST

  • 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
    目的:本研究旨在探讨超声和临床特征联合应用于恶性周围神经鞘瘤(MPNST)和良性周围神经鞘瘤(BPNST)的鉴别诊断,并比较超声和磁共振成像(MRI)的疗效。
    方法:这项回顾性研究包括28个MPNST和57个BPNST的对照组。所有患者都使用LogiqE9(GEHealthCare,密尔沃基,WI)或EPIQ7设备(飞利浦医疗系统,Bothell,西澳)。3.0T核磁共振机(Ingenia;飞利浦医疗,最好的,荷兰)用于扫描,根据患者的临床情况,对不同区域进行常规MRI检查。评估了以下变量:可触知的肿块,疼痛,神经症状,最大直径,location,形状,边界,封装,回声,回声均匀性,存在囊性成分,钙化,目标标志,后回声,和肿瘤的肿瘤间血管。比较超声与临床因素的诊断效能。还评估了预测MPNST与BPNST的独立因素。
    结果:位置参数,形状,边界,封装,MPNST和BPNST之间的血管分布显着不同。多因素logistic回归分析表明,边界,血管分布是MPNSTs的独立预测因子。敏感性,特异性,和尤登指数的三个临床和超声因素(形状,边界,和血管分布)分别为0.89、0.81和0.69,而MRI分别为0.71、0.89和0.61。三种临床和超声综合因素的曲线下面积(AUC)与MRI无明显差异(P>0.05)。
    结论:MRI对MPNSTs和BPNSTs的鉴别诊断有用。然而,结合临床和超声诊断可以达到与MRI相同的效果,包括形状,边界,和脉管系统。
    OBJECTIVE: This study aimed to investigate the combined use of ultrasonography and clinical features for the differentiation of malignant peripheral nerve sheath tumors (MPNST) from benign peripheral nerve sheath tumors (BPNST) and to compare the efficacy of ultrasonography with that of magnetic resonance imaging (MRI).
    METHODS: This retrospective study included 28 MPNSTs and a control group of 57 BPNSTs. All patients underwent an ultrasound scan using the Logiq E9 (GE Health Care, Milwaukee, WI) or EPIQ7 equipment (Philips Medical System, Bothell, WA). A 3.0-T MRI machine (Ingenia; Philips Healthcare, Best, the Netherlands) was used for scanning, and conventional MRI was performed on different regions based on the patient\'s clinical situation. The following variables were evaluated: palpable mass, pain, nerve symptoms, maximum diameter, location, shape, boundary, encapsulation, echogenicity, echo homogeneity, presence of a cystic component, calcification, target sign, posterior echo, and intertumoral vascularity of the tumors. The diagnostic efficacy of ultrasonography and clinical factors was compared with that of MRI. Independent factors for predicting MPNST versus BPNST were also assessed.
    RESULTS: The parameters of location, shape, boundary, encapsulation, and vascularity were significantly different between MPNSTs and BPNSTs. Multiple logistic regression analysis showed that shape, boundary, and vascularity were independent predictors of MPNSTs. The sensitivity, specificity, and Youden index of the three clinical and ultrasound factors (shape, boundary, and vascularity) were 0.89, 0.81, and 0.69, respectively, whereas those of MRI were 0.71, 0.89, and 0.61, respectively. No significant differences in the area under the curve (AUC) of the three combined clinical and ultrasound factors and those of MRI were found (P > .05).
    CONCLUSIONS: MRI was useful in the differential diagnosis between MPNSTs and BPNSTs. However, the combination of clinical and ultrasound diagnoses can achieve the same effect as MRI, including shape, boundary, and vasculature.
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  • 文章类型: Case Reports
    Benign Peripheral Nerve Sheath Tumours (BPNSTs) are traditionally classified into schwannoma, neurofibroma and perinurioma. Due to advances in molecular techniques, hybrid BPNSTs containing more than one histologic types have been documented. Recent studies have demonstrated their frequent association with inherited syndromes like schwannomatosis and neurofibromatosis. Intraneural variant of hybrid neurofibroma/schwannoma is yet to be described. Here we report such a case in a 30-year-old male, who presented with a scalp swelling and histology showed intraneural neurofibromatous tumour admixed with schwannoma-like nodules. IHC (immunohistochemistry) showed variable S100 staining in neurofibromatous areas, diffuse S100 staining in schwannoma-like areas and negative EMA staining in the tumour.
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