• Xianwen Hu, Dandan Li, and Jiong Cai.
• Affiliated Hospital of Zunyi Medical University, Department of Nuclear Medicine, Zunyi, China.
• Medicine (Baltimore). 2022 Dec 9; 101 (49): e32189e32189.

AbstractTo present the clinical experience of primary renal Ewing's sarcoma/primitive neuroectodermal tumors (rEWs/PNET) admitted to our hospital and systematically review the published literature. A retrospective analysis was performed on patients with pathologically confirmed renal EWs/PNET (rEWs) in our hospital, and the literature on rEWs published in PubMed and Embase databases before March 1, 2022 was searched for analysis. A total of 337 rEWs were included in the statistical analysis, including 6 cases of our patients and 331 cases published in the literature. The common clinical symptoms of rEWs are abdominal pain, hematuria, abdominal mass and so on. computed tomography (CT) plays an important role in the diagnosis of rEWs, and the typical manifestation is a large heterogeneous soft tissue density mass, with a specific "septum-like" enhancement in contrast-enhanced scan. The 2-year overall survival rate of rEWs was 48%, with a median survival time of 18 months. "Septum-like" enhancement on CT can be used as a relatively specific sign for the differential diagnosis of rEWs from Wilms tumor and neuroblastoma. The maximum diameter of the rEWs was usually greater than 10 cm, the clinical symptoms of weight loss, metastasis at diagnosis, tumor thrombogenesis of renal vein or/and inferior vena cava tumor, and the failure to undergo radical nephrectomy were the factors of poor prognosis. The incidence of primary rEWs is low and the prognosis is poor. Early diagnosis and radical nephrectomy combined with chemotherapy is the key to improve the prognosis of patients, and CT plays an important role in early diagnosis.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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