Liu Bingcheng, Hu Zhiqi
Department of Plastic Surgery, Nanfang Hospital of Southern Medical University, Guangzhou Guangdong, 510515, P. R. China.
Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2024 Oct 15;38(10):1208-1213. doi: 10.7507/1002-1892.202406085.
To summarize the treatment strategies and clinical experiences of 5 cases of giant plexiform neurofibromas (PNF) involving the head, face, and neck.
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.
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.
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 functional and cosmetic results.
总结5例累及头、面、颈部的巨大丛状神经纤维瘤（PNF）的治疗策略及临床经验。
2021年4月至2023年5月，对5例累及头、面、颈部的巨大PNF患者进行治疗，其中男性1例，女性4例，年龄6 - 54岁（平均22.4岁）。所有肿瘤均呈进行性增大，累及颌面区、耳部及颈部等多个区域，对面部外观影响显著。其中3例肿瘤浸润至深部组织，影响发育，4例伴有听力丧失。影像学检查显示，所有5例肿瘤均以浸润性生长方式为主，其中2例和1例还分别表现为浅表性和推压性生长方式。手术采用基于美学单元的逐步精准治疗策略，而非单纯追求单次手术最大限度切除肿瘤。常规术前对肿瘤供血血管进行栓塞以降低出血风险，随后进行肿瘤切除并联合重建手术。
所有5例患者均接受了1 - 3次术前栓塞治疗，未报告术中出血并发症。4例患者术中需要输血。5例患者共进行了10次手术。1例患者因结扎止血导致术后早期皮瓣边缘坏死；然而，其余患者的切口愈合良好，无并发症。所有患者均随访6至36个月，平均随访时间为21.6个月。随访期间未观察到明显的肿瘤复发。
对于累及头、面、颈部的巨大PNF患者，精准治疗策略可有效控制手术风险，提高美学重建水平。该方法通过减少并发症并优化功能和美容效果，提高了整体治疗效果。
