CAJ | 학술논문

目的：探讨首诊于耳鼻咽喉头颈外科神经纤维瘤病（N F ）的诊断和治疗方法。方法回顾性分析23例N F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。
목적：탐토수진우이비인후두경외과신경섬유류병（N F ）적진단화치료방법。방법회고성분석23례N F환자적림상자료，결합문헌탐토해병적림상표현、진단급치료방법。결과술전병리증실8례，술후병리학진위신경섬유류20례。경과28월（2월～10년）수방，사망4례，1례신경섬유류반악성신경초류사우전신전이，1례쌍측은신경류사우제이측술후계발성뇌간출혈，령2례사우신경섬유류악변반전신전이；건재19례중복발3례，기중악변1례。결론대우표천피부형적NF무수치료，단응정기수방；위우이비인후두경부교대종괴혹고립성신경섬유류，차유국부압박증상혹영향공능급미용자，응채취수술절제；여국부발생악변수채취엄범절제，술후여규범방화료。
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 .