颌骨內神经鞘瘤11例临床分析

Intraosseous Neurilemmoma of the Jaw:Report of 11 Consecutive Cases

目的回顾分析11例颌骨内神经鞘瘤的临床资料,旨在提高对疾病的诊断和治疗水平。方法对1993年1月～2002年2月间经治的11例颌骨内神经鞘瘤患者,从临床、病理、免疫组化、影像学检查及治疗手段等方面进行回顾分析。8例良性者行彻底刮治加烧灼术;2例下颌骨恶性者行扩大切除加颈淋巴清扫术;1例上颌骨恶性伴神经纤维瘤病患者行上颌骨扩大切除加中厚皮片修复及颈淋巴清扫术,术后辅以放疗、化疗。结果5例位上颌骨(1例恶性),6例位下颌骨(2例恶性);其X线表现有一定特异性,术后除1例上颌骨恶性者于11个月时死于广泛多发性转移外,其他10例随访7～122月无复发。结论颌骨内神经鞘瘤临床上少见。CT、MRI及穿刺细胞学检查有助于该病的早期诊断;手术仍是最有效的治疗手段,预后一般良好;辅助放疗、化疗与否,尤其是伴发神经纤维瘤病患者无多大价值。

Objective This study retrospectively analyzed the clinical data of11patients with intraosseous neurilemmoma of the jaw,with the purpose of improve its diagnosis and treatment.Methods11patients with intraosseous neurilemmoma of the jaw treated from January1993to February2002were reviewed,including the clinical features,pathological findings,immunohistochemical findings,imaging examinations and treatment methods.8patients with benign lesions were treated with radical curettage and cautery.2patients with mandibular malignant lesions were treated with extended resection and neck dissection.1patient with maxillary malignant lesion concomitant with neurofibrostosis was treated with extensive resection and middle split skin graft and neck dissection,followed by chemotherapy and radiotherapy after surgery.Results The lesions were located in the maxilla in5patients(1was malignant).6patients had mandibular lesions(2were malignant).The X ray findings had some specificity.10patients were followed up for7to122months without recurrence.1patient with maxillary malignant lesion died of multiple metastasis11months after treatment.Conclusions Intraosseous neurilemmoma of the jaw was rarely seen clinically.CT,MRI and fine needle aspiration biopsy were beneficial for early diagnosis.Surgery is still the dominant choice of treatment.The prognosis of the patients was generally good.Adjuvant radiotherapy or chemotherapy had limited value,especially for patients with neurofibrostosis.A novel regime called CYVADICDE was recently invoked much clinical interest.