Abstract：
Hybrid peripheral nerve sheath tumors show combined features of more than one type of conventional benign peripheral nerve sheath tumors. There are few cases reported of hybrid peripheral nerve sheath tumors in the head and neck region. A 68-year-old female patient was referred for evaluation of an oral swelling lasting five years. Intraoral examination revealed a small mobile nodule located in the lower vestibule. The patient underwent excisional biopsy and microscopic evaluation showed typical features of neurofibroma enclosing areas with palisading nuclei compatible with Antoni A pattern, which are seen in schwannomas. These regions showed strong and diffuse immunoreactivity for S100 protein and moderate positivity in the neurofibroma area. CD34 was positive in the neurofibroma area and entrapped axons were positive for neurofilament. The final diagnosis was oral hybrid neurofibroma-schwannoma tumor. Hybrid peripheral nerve sheath tumors, although extremely rare, may arise within the oral cavity. To the best of our knowledge, this is the first neurofibroma-schwannoma tumor reported in the oral cavity. Recognizing hybrid peripheral nerve sheath tumors as a distinct clinicopathological entity is important because they may also be associated with syndromic disorders.
摘要：
混合性周围神经鞘瘤表现出多种常规良性周围神经鞘瘤的综合特征。头颈部混合性周围神经鞘瘤的报道很少。一名68岁的女性患者接受了持续五年的口腔肿胀评估。口内检查发现位于下前庭的小活动结节。患者接受了切除活检和显微镜评估，显示神经纤维瘤封闭区域的典型特征，具有与AntoniA模式相容的栅栏核，可见于神经鞘瘤。这些区域对S100蛋白显示出强而弥散的免疫反应性，并且在神经纤维瘤区域显示出中等阳性。CD34在神经纤维瘤区域为阳性，而截留的轴突对神经丝呈阳性。最终诊断为口腔混合性神经纤维瘤-神经鞘瘤。混合性周围神经鞘瘤，虽然极为罕见,可能出现在口腔内。据我们所知,这是在口腔中报道的第一个神经纤维瘤-神经鞘瘤肿瘤。认识到混合周围神经鞘瘤是一个独特的临床病理实体是重要的，因为它们也可能与综合征相关。
