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[儿童阴茎原始神经外胚层肿瘤/尤因肉瘤:1例报告并文献复习]

[Primitive neuroectodermal tumor/Ewing's sarcoma of the penis in children: a case report and review of the literature].

作者信息

Zhang Da-Wei, Jin Mei, Zhou Chun-Ju, Song Hong-Cheng, Ma Xiao-Li

机构信息

Department of Hematology, Beijing Children's Hospital Affiliated to Capital University of Medical Sciences, Beijing 100045, China.

出版信息

Zhonghua Nan Ke Xue. 2012 Dec;18(12):1115-8.

Abstract

OBJECTIVE

To investigate the clinical manifestations, pathological characteristics and treatment of primitive neuroectodermal 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 fluorescence in situ hybridization on molecular biological examination. The tumor was confirmed to be PNET/EWS of the penis, and disappeared 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.

摘要

目的

探讨儿童阴茎原始神经外胚层肿瘤/尤文肉瘤(PNET/EWS)的临床表现、病理特征及治疗方法。

方法

分析1例PNET/EWS患儿的临床资料,并复习相关文献。

结果

该患者为5岁男孩,因阴茎肿胀伴疼痛11个月入院。活检显示为小圆细胞肿瘤,免疫组化染色CD99阳性,分子生物学检查荧光原位杂交显示EWS易位。肿瘤确诊为阴茎PNET/EWS,经45周化疗及局部放疗后消失。

结论

阴茎PNET/EWS是一种极其罕见的疾病,除阴茎肿大伴疼痛外无特异性临床症状。免疫组化和分子生物学检查有助于其诊断。

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