综述破裂性神经纤维瘤病1型（NF1）相关巨大丛状神经纤维瘤（PNF）的应急处理和围手术期策略，为改善巨大PNF患者的治疗效果和生活质量提供系统的治疗方案。总结巨大PNF破裂出血的临床经验，总结其诊断、治疗及围手术期处理，实施早期诊断、影像学评估、急诊超选择性动脉栓塞联合手术的一体化诊治策略。手术切除后，可以有效控制急性出血，同时还可以降低术中大出血的风险，最大限度地减少术后并发症。因此，该方法显着提高了治疗成功率和患者生活质量。对于破裂的NF1相关巨大PNF，在多学科团队的协作下，采用紧急超选择性动脉栓塞联合手术切除，可以有效提高治疗成功率率，快速控制出血，缩小肿瘤大小，并降低死亡率。未来的研究应侧重于评估巨大 PNF 破裂出血患者的长期生活质量，并进一步优化治疗方案。

To review the emergency management and perioperative strategies for ruptured neurofibromatosis type 1 (NF1)-related giant plexiform neurofibroma (PNF), providing a systematic treatment protocol to improve the therapeutic outcomes and quality of life for patients with giant PNF.The literature on the management of giant PNF rupture and hemorrhage was reviewed, and the diagnosis, treatment, and perioperative management were summarized based on clinical experiences.By implementing an integrated diagnostic and treatment strategy that includes early diagnosis, imaging evaluation, emergency ultra-selective arterial embolization combined with surgical excision, acute hemorrhage can be effectively controlled while also reducing the risk of major intraoperative bleeding and minimizing postoperative complications. As a result, this approach significantly improves treatment success rates and patient quality of life.For ruptured NF1-related giant PNF, employing emergency ultra-selective arterial embolization combined with surgical excision, under the collaboration of a multidisciplinary team, can effectively improve treatment success rates, rapidly control bleeding, reduce tumor size, and lower mortality. Future research should focus on assessing the long-term quality of life of patients treated for ruptured and hemorrhaging giant PNF and on further optimizing treatment protocols.