下颌骨恶性外周神经鞘膜瘤的临床及病理特点——1例报告及文献复习

Malignant Peripheral Nerve Sheath Tumor of the Mandible:A Case Report and Review of Literature

目的探讨下颌骨恶性外周神经鞘膜瘤的临床及病理特点。方法对1例下颌骨恶性外周神经鞘膜瘤患者的临床资料进行回顾性分析,结合文献复习,探讨其临床特征、组织病理学与影像学特点、治疗和预后。结果该例患者为中年男性,肿物位于左下颌骨区,大小约5cm×6cm,左下唇麻木进行性加重。病理学为肉瘤表现。影像学特点为左下牙槽神经管局部扩大,周围骨质密度较健侧低。行左下颌骨切除、左舌骨上淋巴清扫加肋骨钛板重建术,术后患者接受辅助性放疗45d,共30次,总量60Gy,放疗过程顺利。术后半年肺部转移。结论下颌骨恶性外周神经鞘膜瘤为恶性病变,临床少见,确诊依据病理和免疫组化,治疗以手术为主,预后较差。

Objective To report one case of malignant peripheral nerve sheath tumor of mandible with review of literatures thus to investigate its clinical and pathological characteristics.Methods One case of malignant peripheral nerve sheath tumor of mandible was analyzed retrospectively and the relative literature were reviewed to investigate clinical andhistopathological features,the photography and treatments.Results The case of malignant peripheral nerve sheath tumor of mandible was in young male.The tumor was in the left lower jaw.It was about 5 cm ×6 cm.The lip is gradual numb,and the histological features of the tumor was sarcoma.The photographic features of the tumor showed that the canal of the lower alveolar nerve was extend partly and the consistency of the bone was lower than the healthy one.Conclusion With review of relative literatures,malignant peripheral nerve sheath tumor of mandible is a malignant tumor and it is scarcely and the main method of treatment is surgical operation,the definite diagnosis depends on the pathological and the immunohistochemical staining.