小儿巨大阴茎神经纤维瘤1例并文献复习

The giant neurofibroma of penis in a child： a case report and literature review

目的 探讨小儿巨大阴茎神经纤维瘤的临床表现、病理特点、诊断及鉴别诊断、治疗方法和预后.方法 回顾性分析广州市妇女儿童医疗中心2012年2月收治的1例巨大阴茎神经纤维瘤患儿的临床资料（一般资料、影像学资料、治疗方法、病理检查结果及预后等）并对相关文献进行复习.结果 阴茎肿瘤肉眼大体所见为一大小约9cm×11 cm×15 cm的实体肿瘤,局部破溃.CT显示阴茎体可见一大小约9.0 cm×10.0cm×13.4cm巨大肿物.行阴茎部分切除术,术后残留阴茎体长约2 cm.病理检查结果：HE染色梭形肿瘤细胞弥散分布,部分胞质红染,核呈梭形或椭圆形,部分呈波浪状;免疫组织化学染色显示S-100及波形蛋白均呈阳性,提示为神经纤维瘤.术后随访2年肿瘤未复发,患儿阴茎有正常的感觉,并且具有勃起功能.结论 小儿阴茎神经纤维瘤极为罕见,要注意与导致阴茎肿大的其他疾病相鉴别.可选择个体化手术方式,完整切除肿瘤是首选的手术方式,尽量争取达到功能及外观恢复的最佳效果.

Objective To investigate the clinical and pathological characteristics,diagnosis,differential diagnosis,treatment and prognosis of giant neurofibroma of penis in the child.Methods The clinical data including general data,imaging data,treatment methods,pathological characteristics of a case with giant neurofibroma of penis in a child were analyzed retrospectively and the relevant literature was reviewed.Results Gross appearance of the penile shaft neurofibroma was about 9 cm × 11 cm × 15 cm,with local ulceration.Computerized tomography scan revealed a giant mass in the penile shaft,about 9.0 cm × 10.0 cm × 13.4 cm.Partial excision of the penis was performed.Postoperative appearance of the residual penile shaft was about 2 cm long.The HE staining showed spindle cells with the red dye cytoplasm,spindle or elliptic nuclei and arranged in wavy partly.Positive immunostaining was presented with S-100 protein and Vimentin.The pathologic examination revealed a neurofibroma.There was no evidence of recurrence and the penis of the boy had normal sensation and erection by follow-up in 2 years.Conclusions Neurofibroma of penis in the child is extremely rare and the differential diagnosis of soft-tissue tumors of penis should be considered.The operative method should be individualized,the treatment goal is the complete resection;however,this goal must be weighed against detriment to functioning and the cosmetics of the involved organ.