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先天性尤因肉瘤/外周原始神经外胚层肿瘤:一例报告并文献复习

Congenital Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature.

作者信息

Jin Shu-Guang, Jiang Xiao-Ping, Zhong Lin

机构信息

Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu 610041, China.

Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu 610041, China.

出版信息

Pediatr Neonatol. 2016 Oct;57(5):436-439. doi: 10.1016/j.pedneo.2013.11.002. Epub 2014 Jan 27.

Abstract

Ewing's sarcoma (EWS) and peripheral primitive neuroectodermal tumor (pPNET) are small round cell malignancies that develop in soft tissue and bone. They very rarely affect newborns. A diagnosis of EWS/pPNET depends mainly on immunohistochemistry and molecular/genetic assays. Since these tumors are highly aggressive, patient prognosis is typically very poor, and treatment remains a challenge. Here, we report a 13-day-old newborn diagnosed with congenital EWS/pPNET and describe its treatment.

摘要

尤因肉瘤(EWS)和外周原始神经外胚层肿瘤(pPNET)是发生于软组织和骨骼的小圆细胞恶性肿瘤。它们极少影响新生儿。EWS/pPNET的诊断主要依赖免疫组织化学和分子/基因检测。由于这些肿瘤具有高度侵袭性,患者预后通常很差,治疗仍然是一项挑战。在此,我们报告一名13天大的新生儿被诊断为先天性EWS/pPNET,并描述其治疗情况。

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