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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 features and treatment of primitive neuroectodermal tumor (PNET / Ewing’s sarcoma) in the penis. Methods: We reviewed the literature of one case of PNET / EWS in our hospital who had a 5-year-old child with primary cystic penis. Results: The pathology showed small cell malignant tumor. Immunohistochemistry showed that CD99 (+), molecular biology examination: EWS fluorescent in situ hybridization showed translocation, which was in accordance with the diagnosis of primitive neuroectodermal tumor / Ewing’s sarcoma. Pathology diagnosed as penile PNET / EWS, after 45 weeks of chemotherapy and local radiotherapy, tumor shrinkage. Currently discontinued for 3 months. Conclusion: The primary penile PNET / EWS is extremely rare and the clinical symptoms are nonspecific. Penile enlargement and pain predominates. Immunohistochemistry and molecular biology are helpful for the diagnosis of PNET / EWS.