眼眶丛状神经纤维瘤合并腹膜后神经纤维瘤1例报告并文献复习

A Case Report and Literature Review of Orbital Plexiform Neurofibroma Complicated with Retroperitoneal Neurofibroma

摘要: 目的：探讨眼眶丛状神经纤维瘤的临床特征，以加深对该肿瘤的认识。方法：回顾性分析1例眼眶丛状神经纤维瘤患者的临床资料，并结合国内外文献进行复习探讨。结果：患者以发现左眼眼球突出2年为主要临床表现，眼科联合神经外科经颅开眶行眶内及颅内肿瘤切除术，术后病理学检查示肿瘤细胞排列松散，呈纺锤样，组织可见丰富小血管及绳索样的粗大神经，神经纤维束呈波浪状排列。免疫组化染色示S-100阳性、SOX10弱阳性，不表达SMA、EMA、CD34。随访1个月未见肿瘤复发、转移。结论：眼眶丛状神经纤维瘤临床较罕见，具有独特的病理学特征，手术治疗疗效确切。

Abstract: Objective: To investigate the clinical features of plexiform neurofibroma (PNF) to improve under-standing. Methods: The clinical data of a patient with orbital plexiform neurofibroma were retro-spectively described, reviewed and discussed with domestic and international literature. Results: The patient presented with eye tubercle for 2 years as a main clinical manifestation. Ophthalmology combined with neurosurgery performed transcranial orbital and intracranial tumor resection. The tumor cells were loosely arranged and spindle-like, with abundant small blood vessels and rope-like thick nerves visible in the tissues. The nerve fiber bundles were arranged in a wavy pat-tern. Immunohistochemistry showed that the tumor cells were positive for S-100, weakly positive for SOX10, and didn’t express SMA, EMA, CD34. No tumor recurrence or metastasis was observed in the 1-month follow-up after surgery. Conclusion: Orbital plexiform neurofibroma is rare clinically, and has unique clinical pathology features. The efficacy of surgical excision is definite.