精准化治疗五例头面颈部巨大丛状神经纤维瘤的临床经验  

Clinical experiences in precision treatment of giant plexiform neurofibromas of head,face,and neck

作　　者：刘秉承[1] 胡志奇[1] LIU Bingcheng;HU Zhiqi(Department of Plastic Surgery,Nanfang Hospital of Southern Medical University,Guangzhou Guangdong,510515,P.R.China)

机构地区：[1]南方医科大学南方医院整形外科,广州510515

出　　处：《中国修复重建外科杂志》2024年第10期1208-1213,共6页Chinese Journal of Reparative and Reconstructive Surgery

基　　金：国家自然科学基金资助项目(82172235);中国博士后科学基金资助项目(2022M711512);南方医科大学南方医院院长基金(2020Z003)。

摘　　要：目的总结5例头面颈部巨大丛状神经纤维瘤(plexiform neurofibromas,PNF)的治疗策略和临床经验。方法2021年4月-2023年5月,收治5例头面颈部巨大PNF患者。男1例,女4例;年龄6~54岁,平均22.4岁。肿瘤均进行性增大,累及颌面、耳、颈部等多个部位,影响面部外观;其中3例浸润深部组织影响发育、4例合并听力下降。影像学检查示5例肿瘤生长模式均以侵袭型为主,其中2例存在浅表型区域,1例局部表现为移位型。以实现“精准化治疗”为目标,综合瘤体生长模式和所处解剖分区美学要求制定治疗方案。常规行瘤体滋养血管介入栓塞降低手术出血风险,随后行肿瘤切除联合外观重建手术。结果5例患者术前行1~3次血管栓塞,术中均未发生出血意外,其中4例术中接受输血治疗。5例患者共接受10次手术,1例术后早期皮肤切缘因缝扎止血导致缺血坏死;其余患者切口均顺利愈合,无术后并发症发生。患者术后均获随访,随访时间6~36个月,平均21.6个月。随访期间肿瘤均未见明显复发。结论对于头面颈部巨大PNF患者,精准化治疗能有效控制手术风险,提高外观修复精度。Objective To summarize the treatment strategies and clinical experiences of 5 cases of giant plexiform neurofibromas(PNF)involving the head,face,and neck.Methods Between April 2021 and May 2023,5 patients with giant PNFs involving the head,face,and neck were treated,including 1 male and 4 females,aged 6-54 years(mean,22.4 years).All tumors showed progressive enlargement,involving multiple regions such as the maxillofacial area,ear,and neck,significantly impacting facial appearance.Among them,3 cases involved tumor infiltration into deep tissues,affecting development,while 4 cases were accompanied by hearing loss.Imaging studies revealed that all 5 tumors predominantly exhibited an invasive growth pattern,in which 2 and 1 also presenting superficial and displacing pattern,respectively.The surgical procedure followed a step-by-step precision treatment strategy based on aesthetic units,rather than simply aiming for maximal tumor resection in a single operation.Routine preoperative embolization of the tumor-feeding vessels was performed to reduce bleeding risk,followed by tumor resection combined with reconstructive surgery.Results All 5 patients underwent 1-3 preoperative embolization procedures,with no intraoperative hemorrhagic complications reported.Four patients required intraoperative blood transfusion.A total of 10 surgical procedures were performed across the 5 patients.One patient experienced early postoperative flap margin necrosis due to ligation for hemostasis;however,the incisions in the remaining patients healed without complications.All patients were followed up for a period ranging from 6 to 36 months,with a mean follow-up duration of 21.6 months.No significant tumor recurrence was observed during the follow-up period.Conclusion For patients with giant PNF involving the head,face,and neck,precision treatment strategy can effectively control surgical risks and improve the standard of aesthetic reconstruction.This approach enhances overall treatment outcomes by minimizing complications and optimizing fu

关 键 词：Ⅰ型神经纤维瘤病 丛状神经纤维瘤 精准化治疗策略 

分 类 号：R739.91[医药卫生—肿瘤]