首诊于耳鼻咽喉头颈外科神经纤维瘤病的诊疗体会  

The Diagnosis and Treatment of Neurofibromatosis Firstly-diagnosed in Otolaryngology Head and Neck Surgery

作　　者：黄建强[1] 黄方[1] 黄斯诚[2] 吴春林[3] 黄文瀚[4] 胡娟娟[1] 

机构地区：[1]福建医科大学附属第二医院耳鼻咽喉头颈外科,泉州362000 [2]中南大学湘雅医学院临床医学系 [3]福建医科大学附属第二医院病理科,泉州362000 [4]福建医科大学附属第二医院放射科,泉州362000

出　　处：《福建医科大学学报》2014年第4期248-252,共5页Journal of Fujian Medical University

摘　　要：目的探讨首诊于耳鼻咽喉头颈外科神经纤维瘤病(NF)的诊断和治疗方法。方法回顾性分析23例NF患者的临床资料,结合文献探讨该病的临床表现、诊断及治疗方法。结果术前病理证实8例,术后病理确诊为神经纤维瘤20例。经过28月(2月~10年)随访,死亡4例,1例神经纤维瘤伴恶性神经鞘瘤死于全身转移,1例双侧听神经瘤死于第二侧术后继发性脑干出血,另2例死于神经纤维瘤恶变伴全身转移;健在19例中复发3例,其中恶变1例。结论对于表浅皮肤型的NF无须治疗,但应定期随访;位于耳鼻咽喉头颈部较大肿块或孤立性神经纤维瘤,且有局部压迫症状或影响功能及美容者,应采取手术切除;如局部发生恶变须采取广泛切除,术后予规范放化疗。Objective To investigate the diagnosis and treatment of neurofibromatosis （NF）firstly-diagnosed in otolaryngology head and neck surgery . Methods The clinical data of 23 patients with NF which was received from 1990 February to 2014 May were retrospectively analyzed . The clinical presen-tation、diagnosis and management were discussed combined with literatures review . Results 8 cases were conducted preoperative pathology ,20 cases were neurofibroma by postoperative pathology diagnosis . Af-ter follow-up for 28 months （2 months^10 years） ,4 cases were dead ,1 case died of systemic metastasis of neurofibromas with malignant schwannoma ,1 case which was bilateral acoustic neuroma died of brain stem hemorrhage after the second operation ,the other 2 cases died of neurofibroma with malignant transforma-tion and systemic metastasis ;19 cases were still alive ,among them ,3 cases occurred recurrence ,including 1 case malignant transformation . Conclusion Superficial skin type multiple neurofibroma just only need observation with regular follow-up . Larger tumor in otolaryngology head and neck or solitary neurofibro-ma w hich has local compression symptoms or impact function and hairdressing ,should be conducted surgi-cal resection . If malignant transformation appears ,wide excision assisted with postoperative standard ra-diotherapy and chemotherapy is a better choice .

关 键 词：神经纤维瘤病 神经鞘肿瘤 头部 颈 外科手术 

分 类 号：R739.4[医药卫生—肿瘤]