Abstract：
BACKGROUND: Giant neurofibromas occurring in individuals diagnosed with neurofibromatosis type 1 (NF1) often result in considerable disfigurement, functional impairment, and diminished quality of life. Although debulking surgery poses inherent risks of complications, it remains the most efficacious approach to address these issues. The primary objective of this study was to share our surgical experience with giant neurofibromas in the extremities and trunk wall of NF1 patients which may help surgeons to minimize intraoperative bleeding and facilitate tumor excision.
METHODS: A retrospective review was conducted at a single center, encompassing 36 NF1 patients with giant neurofibromas in the extremities and trunk wall who underwent debulking surgery from July 2010 to July 2022.
RESULTS: Twenty-one male and fifteen female NF1 patients who received one to four surgical interventions were evaluated. The average age at the time of surgery was 17.8 years. The median follow-up time was 52 months. Our findings revealed relatively low rates of complications and recurrence. Notably, patients expressed satisfaction with both the aesthetic and functional results.
CONCLUSIONS: Debulking surgery of giant neurofibromas in the extremities and trunk wall of NF1 patients can effectively reduce the tumor burden, leading to improvements in both the appearance and function.
METHODS: A retrospective review was conducted at a single center, encompassing 36 NF1 patients with giant neurofibromas in the extremities and trunk wall who underwent debulking surgery from July 2010 to July 2022.
RESULTS: Twenty-one male and fifteen female NF1 patients who received one to four surgical interventions were evaluated. The average age at the time of surgery was 17.8 years. The median follow-up time was 52 months. Our findings revealed relatively low rates of complications and recurrence. Notably, patients expressed satisfaction with both the aesthetic and functional results.
CONCLUSIONS: Debulking surgery of giant neurofibromas in the extremities and trunk wall of NF1 patients can effectively reduce the tumor burden, leading to improvements in both the appearance and function.
摘要：
背景：在诊断为1型神经纤维瘤病（NF1）的个体中发生的巨大神经纤维瘤通常会导致相当大的毁容，功能损害，生活质量下降。尽管减积手术会带来固有的并发症风险，它仍然是解决这些问题的最有效方法。这项研究的主要目的是分享我们在NF1患者四肢和躯干壁的巨大神经纤维瘤的手术经验，这可能有助于外科医生减少术中出血并促进肿瘤切除。
方法：在单个中心进行了回顾性审查，2010年7月至2022年7月，纳入36例NF1四肢和躯干壁巨大神经纤维瘤患者。
结果：对21名男性和15名女性NF1患者进行了评估，这些患者接受了1至4次手术干预。手术时的平均年龄为17.8岁。中位随访时间为52个月。我们的发现显示并发症和复发率相对较低。值得注意的是,患者对美学和功能结果表示满意。
结论：NF1患者四肢和躯干壁巨大神经纤维瘤的膨化手术可有效减轻肿瘤负担，导致外观和功能的改进。
方法：在单个中心进行了回顾性审查，2010年7月至2022年7月，纳入36例NF1四肢和躯干壁巨大神经纤维瘤患者。
结果：对21名男性和15名女性NF1患者进行了评估，这些患者接受了1至4次手术干预。手术时的平均年龄为17.8岁。中位随访时间为52个月。我们的发现显示并发症和复发率相对较低。值得注意的是,患者对美学和功能结果表示满意。
结论：NF1患者四肢和躯干壁巨大神经纤维瘤的膨化手术可有效减轻肿瘤负担，导致外观和功能的改进。
