目的 头颈部丛状神经纤维瘤(PNF)的治疗是临床难题，在手术治疗、分类、治疗时机和治疗方法上缺乏共识。通过对头颈部PNF手术治疗患者的临床表现、手术情况、肿瘤复发情况、治疗满意度及生活质量改变的分析，为进一步形成共识提供依据。 方法 通过病历查阅及电话随访的方式，回顾性分析2012年5月至2022年7月上海交通大学医学院附属第九人民医院整复外科因头颈部PNF进行手术治疗的Ⅰ型神经纤维瘤病(NF1)患者资料。完整收集、统计分析患者的临床资料，采用电话随访方式对患者和(或)家属的术后即刻满意度、远期手术满意度，以及标准化生活质量问卷HRQol和PlexiQol进行调查。根据治疗前后生活质量改变和远期手术满意度，将患者分为手术获益组及非获益组，使用单因素及多因素logistic回归分析远期手术获益患者的临床特点。 结果 有完整记录的头颈部NF1入院手术患者共计512例，随访时筛选病史及影像学明确为NF1相关PNF，并取得有效随访的121例患者纳入研究。男70例，女51例，年龄(25.60±12.85)岁，范围7～63岁，其中≤18岁的患者41例，>18岁的患者80例。瘤体以侵袭性生长为主，62.81%(76/121)患者表现出临床功能障碍。41.32%(50/121)患者接受了多次手术治疗，121例患者总计进行了215次手术。手术目的包括改善外观及功能修复，术后并发症发生率为6.05%(13/215)。末次术后随访时间为(51.41±27.66)个月，42.15%(51/121)患者表示术后瘤体有复发。对术后即刻效果比较满意及非常满意的患者占76.03%(92/121)，但在远期随访时满意率降至46.28%(56/121)。≤18岁的患者家属对手术造成的瘢痕不满意比例更高，且有更强的再次手术意愿。瘤体复发与否和手术获益密切相关(OR＝2.32，P<0.05)。进一步分析发现年龄及性别是头颈部PNF复发的危险因素，其中≤18岁的患者复发风险显著高于>18岁患者(OR＝3.49, P＝0.004)，并且7～12岁年龄段患者的末次术后复发率最高，达到68.42%(13/19)；男性患者的复发概率显著低于女性(OR＝0.40, P＝0.026)。 结论 头颈部PNF患者临床表现复杂，临床诊疗应注重充分的术前评估、积极的多学科诊疗协作和综合应用包括手术及靶向药物的治疗方法，以提高安全性及有效性，减少瘤体复发。 Objective The treatment of head and neck in plexiform neurofibroma (PNF) is a major clinical problem, lacking consensus on surgical treatment, classification, operation timing, and treatment method. The purpose of this study was to provide a basis for further consensus formation by analyzing the clinical manifestations, surgical conditions, tumor recurrence, post-operation satisfaction, and changes in quality of life of patients undergoing PNF surgery in head and neck. Methods Through medical record review and telephone follow-up, a retrospective analysis was conducted on neurofibromatosis type 1 (NF1) patients admitted for surgical treatment for PNF patient in head and neck from May 2012 to July 2022 in Department of Plastic and Reconstructive Surgery, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine. Complete collection and statistical analysis of patients’clinical data, using telephone follow-up to investigate the immediate postoperative satisfaction and long-term surgical satisfaction of patients and/or their families, as well as standardized quality of life questionnaires HRQol(health related quality of life) and PlexiQol(plexiform neurofibroma quality of life). Based on the data about changes in quality of life before and after surgery and long-term surgical satisfaction, patients were divided into surgical benefit and non-benefit groups. Binary and multivariate logistic regression analysis were used to analyze the clinical characteristics of patients with long-term surgical benefit. Results Totally 512 patients with head and neck NF1 were admitted for surgery with complete medical records. 121 patients were identified as NF1 related PNF diagnosed by medical history and radiological examination, and effective follow-up was obtained. There were 70 males and 51 females, aged (25.60±12.85) years old, ranging from 7 to 63 years old, with 41 patients who were ≤ 18 years old and 80 patients over 18 years old. 62.81%(76/121) of patients exhibiting clinical dysfunctions, and the tumor mass were mainly characterized by invasive growth. 41.32%(50/121) of patients underwent multiple surgical treatments, with a total of 215 surgeries performed on 121 patients. The surgical objective included appearance improvement and functional repair. The incidence of postoperative complications was 6.05%(13/215). The follow-up period after last operation was (51.41±27.66) months, and 42.15%(51/121) of patients reported postoperative tumor recurrence. 76.03%(92/121) of patients were satisfied with immediate postoperative result, while the rate decreased to 46.28%(56/121) during long-term follow-up. Family members of patients who were ≤ 18 years old had a higher proportion of dissatisfaction with the scars caused by surgery and a stronger willingness to undergo another surgery. The tumor recurrence was closely related to surgical benefits (OR=2.32, P<0.05). Further analysis found that the gender and age of patients were the main risk factors for the recurrence. The recurrence risk in patients ≤ 18 years old was significantly higher than in that over 18 years old(OR=3.49, P=0.004), and the highest in the 7-12 year-old group, reaching 68.42%(13/19). The recurrence risk in male patients was significantly lower than that in females (OR=0.40, P=0.026). Conclusion The clinical manifestations of PNF patients in head and neck region are complex. Clinical diagnosis and treatment in PNF should focus on the applications in comprehensive method such as full preoperative evaluation, active multi-disciplinary treatment cooperation and combined therapies in order to improve the safety and effectiveness of treatment and reduce tumor recurrence.
Retrospective study of 121 patients with plexiform neurofibroma in head and neck
Objective The treatment of head and neck in plexiform neurofibroma (PNF) is a major clinical problem, lacking consensus on surgical treatment, classification, operation timing, and treatment method. The purpose of this study was to provide a basis for further consensus formation by analyzing the clinical manifestations, surgical conditions, tumor recurrence, post-operation satisfaction, and changes in quality of life of patients undergoing PNF surgery in head and neck. Methods Through medical record review and telephone follow-up, a retrospective analysis was conducted on neurofibromatosis type 1 (NF1) patients admitted for surgical treatment for PNF patient in head and neck from May 2012 to July 2022 in Department of Plastic and Reconstructive Surgery, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine. Complete collection and statistical analysis of patients’clinical data, using telephone follow-up to investigate the immediate postoperative satisfaction and long-term surgical satisfaction of patients and/or their families, as well as standardized quality of life questionnaires HRQol(health related quality of life) and PlexiQol(plexiform neurofibroma quality of life). Based on the data about changes in quality of life before and after surgery and long-term surgical satisfaction, patients were divided into surgical benefit and non-benefit groups. Binary and multivariate logistic regression analysis were used to analyze the clinical characteristics of patients with long-term surgical benefit. Results Totally 512 patients with head and neck NF1 were admitted for surgery with complete medical records. 121 patients were identified as NF1 related PNF diagnosed by medical history and radiological examination, and effective follow-up was obtained. There were 70 males and 51 females, aged (25.60±12.85) years old, ranging from 7 to 63 years old, with 41 patients who were ≤ 18 years old and 80 patients over 18 years old. 62.81%(76/121) of patients exhibiting clinical dysfunctions, and the tumor mass were mainly characterized by invasive growth. 41.32%(50/121) of patients underwent multiple surgical treatments, with a total of 215 surgeries performed on 121 patients. The surgical objective included appearance improvement and functional repair. The incidence of postoperative complications was 6.05%(13/215). The follow-up period after last operation was (51.41±27.66) months, and 42.15%(51/121) of patients reported postoperative tumor recurrence. 76.03%(92/121) of patients were satisfied with immediate postoperative result, while the rate decreased to 46.28%(56/121) during long-term follow-up. Family members of patients who were ≤ 18 years old had a higher proportion of dissatisfaction with the scars caused by surgery and a stronger willingness to undergo another surgery. The tumor recurrence was closely related to surgical benefits (OR=2.32, P<0.05). Further analysis found that the gender and age of patients were the main risk factors for the recurrence. The recurrence risk in patients ≤ 18 years old was significantly higher than in that over 18 years old(OR=3.49, P=0.004), and the highest in the 7-12 year-old group, reaching 68.42%(13/19). The recurrence risk in male patients was significantly lower than that in females (OR=0.40, P=0.026). Conclusion The clinical manifestations of PNF patients in head and neck region are complex. Clinical diagnosis and treatment in PNF should focus on the applications in comprehensive method such as full preoperative evaluation, active multi-disciplinary treatment cooperation and combined therapies in order to improve the safety and effectiveness of treatment and reduce tumor recurrence.
Neurofibroma, plexiformNeurofibromatosis type 1Head and neck neoplasmsTumor recurrence
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