儿童阴茎原始神经外胚层肿瘤／尤文氏瘤1例并文献复习  

Primitive neuroectodermal tumor/Ewing's sarcoma of the penis in children: A case report and review of the literature

作　　者：张大伟[1] 金眉[1] 周春菊[2] 宋宏程[3] 马晓莉[1] 

机构地区：[1]首都医科大学附属北京儿童医院 血液病中心 [2]首都医科大学附属北京儿童医院 病理科 [3]首都医科大学附属北京儿童医院 泌尿外科,北京100045

出　　处：《中华男科学杂志》2012年第12期1115-1118,共4页National Journal of Andrology

摘　　要：目的:探讨原发于阴茎的原始神经外胚层肿瘤/尤文氏瘤(PNET/Ewing's sarcoma)的临床表现、病理特点、治疗方法。方法:分析本院收治的1例5岁患儿原发于阴茎的PNET/EWS患者的病例资料进行文献复习。结果:病理表现为小圆细胞恶性肿瘤,免疫组化结果显示CD99(+),分子生物学检查:EWS荧光原位杂交可见易位,符合原始神经外胚层肿瘤/尤文氏肉瘤诊断,该患者经过病理确诊为阴茎PNET/EWS,经45周化疗和局部放疗,瘤灶缩小。目前停药3个月。结论:原发阴茎PNET/EWS极罕见,临床症状无特异性,以阴茎增大伴疼痛为主,免疫组化及分子生物学检查有助于对PNET/EWS的确诊。Objective： To investigate the clinical manifestations, pathological characteristics and treatment of primitive neuroec- todermal tumor/ Ewing＇s sarcoma （PNET/EWS） of the penis in children. Methods： We analyzed the clinical data of a case of PNET/EWS and reviewed relevant literature. Results ： The patient was a 5-year-old boy, admitted for penis swelling with pain for 11 months. Biopsy showed a small round cell tumor, CD99 positive by immunohistochemical staining, with EWS translocation by fluores- cence in situ hybridization on molecular biological examination. The tumor was confirmed to be PNET/EWS of the penis, and disap- peared after 45 weeks of chemotherapy and local radiotherapy. Conclusion ： PNET / EWS of the penis is an extremely rare disease, with no specific clinical symptoms except penis enlargement with pain. Immunohistochemistry and molecular biological examination contribute to its diagnosis.

关 键 词：阴茎肿瘤 原始神经外胚层肿瘤 尤文氏瘤 

分 类 号：R737.27[医药卫生—肿瘤]