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Renal Ewing sarcoma/primitive neuroectodermal tumor in a pregnant woman who underwent robot-assisted laparoscopic nephrectomy: a case report and literature review

Authors Miao C, Yang J, Xue J, Zhu J, Chen W, Qin Y, Wang Z

Received 30 October 2017

Accepted for publication 7 March 2018

Published 11 October 2018 Volume 2018:11 Pages 6839—6843

DOI https://doi.org/10.2147/OTT.S155523

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Cristina Weinberg

Peer reviewer comments 2

Editor who approved publication: Dr Yao Dai


Chenkui Miao,1,* Jie Yang,1,* Jianxin Xue,1,* Jundong Zhu,1 Wen Chen,2 Yuan Qin,1 Zengjun Wang1

1State Key Laboratory of Reproductive Medicine and Department of Urology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, People’s Republic of China; 2Department of Pathology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, People’s Republic of China

*These authors contributed equally to this work

Abstract: Primary Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) of the kidney represents a spectrum of rare neoplasm with dismal clinical prognosis. This type of malignant tumor predominantly occurs in the soft tissue and bones of pediatric–young adults, and it may rarely arise from the kidney. Derived from the neuroectoderm, renal ES/PNET belongs to a group of primitive and aggressive tumors in its biological manifestation. Herein, we report the case of a 40-year-old pregnant woman with renal mass, in whom was found gross hematuria and slight lumbar acid during pregnancy. A computed tomography scan revealed an irregular soft tissue mass approximately 5×5×5 cm in size. The patient underwent robot-assisted laparoscopic nephrectomy of the right kidney after childbirth. The diagnosis of renal ES/PNET was confirmed by immunohistochemical detection and fluorescence in situ hybridization of the nephrectomy specimen. Primary renal ES/PNET represents a rare and lethal entity, especially in a 40-year-old pregnant woman. Although the clinical presentation of this tumor is nonspecific, renal ES/PNET frequently exert dismal prognosis and aggressive clinical outcomes. Thus, it is essential to distinguish ES/PNET from other renal cell carcinomas and carry out an optimum treatment strategy as soon as possible.

Keywords: Ewing sarcoma, primitive neuroectodermal tumor, kidney, pregnancy, robot-assisted surgery

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