11例头颈部Ⅰ型神经纤维瘤病的临床分析

Clinical analysis of 11 cases of type Ⅰ head and neck neurofibromatosis

目的探讨头颈部Ⅰ型神经纤维瘤病的临床表现、诊断及治疗方法。方法收集11例头颈部Ⅰ型神经纤维瘤病患者的临床资料。回顾性分析该病的临床表现、诊断、治疗方法等。结果 11例Ⅰ型神经纤维瘤病头颈部手术均取得成功,术中出血量300ml^1500ml,未发生失血性休克病例,转移皮瓣均一期成活。11例患者病理切片均为I型神经纤维瘤病。6例肿瘤无明显包膜,5例可见不完整包膜。其中局限型2例(18.2%),弥漫型7例(63.6%),丛状型2例(18.2%)。全部病例随访0.5a^6a未见复发。结论头颈部Ⅰ型神经纤维瘤病临床表现多样,诊断须结合临床表现和各种实验室检查不难确诊,在治疗上,手术要根据肿块的具体情况采取一次根除或者二次切除。

Objective To investigate the clinical manifestations, diagnosis and treatment in the head and neck neurofibromatosis（type Ⅰ）. Methods Clinical data of 11 cases of the head and neck neurofibromatosis type I were reviewed. A retrospective analysis of the clinical manifestations of the disease, diagnosis, treatment methods were done. Results One case had a genetic history, two cases of patients had a family history. 8 males and 3 females, aged 12 -41 years, mean age 24.5 ±7.9 years. All cases were operated successfully, blood loss 300ml-1500ml, without hemorrhagic shock, and with the uniformity of skin graft survival, caught flap survival.11 patients were pathological type I neurofibromatosis. Six cases presented no obvious tumor capsule, and seen five cases of incomplete capsule. Among them, limited type was two cases（18.2%）, diffuse type was seven cases（63.6%）, and plexiform type was two cases（18.2%）. All patients were followed up from 0.5a to 6a, and no recurrence was found. Conclusion The clinical manifestations of head and neck neurofibromatosis type I were various, diagnosis should be combined with clinical manifestations and laboratory examination is not difficult to diagnose, in the treatment, surgery should be taken once or twice to eradicate tumor resection depending on the specific circumstances of the masses.