Abstract：
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.
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.
摘要：
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。
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。
