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模仿肾细胞癌并伴有广泛转移的原发性肾脏尤因肉瘤:一例报告及文献简要综述

Primary Ewing's Sarcoma of the Kidney Mimicking Renal Cell Carcinoma With Widespread Metastasis: A Case Report and a Brief Review of the Literature.

作者信息

Rangankar Varsha, Dhande Aryaman, Khaladkar Sanjay M, Kirdat Patil Prajakta P, Bhuibhar Gayatri

机构信息

Radiodiagnosis, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, Pune, IND.

Pathology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, Pune, IND.

出版信息

Cureus. 2024 Jul 19;16(7):e64896. doi: 10.7759/cureus.64896. eCollection 2024 Jul.

DOI:10.7759/cureus.64896
PMID:39156398
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11330583/
Abstract

Ewing's sarcoma family of tumors (ESFTs) are a group of small round cell tumors with common morphological and genetic features, including Ewing's sarcoma of bone, primary extra-skeletal Ewing tumors, extraosseous Ewing sarcoma (EES), and Askin tumors. EES presenting as a primary renal mass is an exceedingly uncommon aggressive tumor with limited reported cases in the literature and often mimics other renal malignancies on imaging. We present a case of a 31-year-old man presenting with left flank pain and abdominal fullness of short duration. Radiological imaging studies showed a large heterogenous mass from the left kidney, confirmed to be Ewing's sarcoma on post-operative histopathological examination (HPE) and immunohistochemistry (IHC) studies. Subsequent follow-up showed extensive metastatic disease. EES of the kidney has a nonspecific presentation and imaging appearance necessitating a multi-disciplinary approach comprising radiological imaging with a high index of suspicion, HPE, IHC, and molecular analysis for the correct diagnosis.

摘要

尤因肉瘤家族性肿瘤(ESFTs)是一组具有共同形态学和遗传学特征的小圆细胞肿瘤,包括骨尤因肉瘤、原发性骨外尤因肿瘤、骨外尤因肉瘤(EES)和阿斯金瘤。表现为原发性肾脏肿块的EES是一种极为罕见的侵袭性肿瘤,文献报道的病例有限,且在影像学上常与其他肾脏恶性肿瘤相似。我们报告一例31岁男性,出现左侧腰痛和短期内腹部胀满。放射学影像学检查显示左肾有一个大的异质性肿块,术后组织病理学检查(HPE)和免疫组织化学(IHC)研究证实为尤因肉瘤。随后的随访显示有广泛的转移性疾病。肾脏EES的表现和影像学表现不具有特异性,需要采用多学科方法,包括高度怀疑的放射学影像学检查、HPE、IHC和分子分析,以做出正确诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/9bfa12d0b7af/cureus-0016-00000064896-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/970b85f72cee/cureus-0016-00000064896-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/067e58d724e2/cureus-0016-00000064896-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/d263916a7431/cureus-0016-00000064896-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/a1c14af29b26/cureus-0016-00000064896-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/f27058d50577/cureus-0016-00000064896-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/73e41be7c4a0/cureus-0016-00000064896-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/9bfa12d0b7af/cureus-0016-00000064896-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/970b85f72cee/cureus-0016-00000064896-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/067e58d724e2/cureus-0016-00000064896-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/d263916a7431/cureus-0016-00000064896-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/a1c14af29b26/cureus-0016-00000064896-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/f27058d50577/cureus-0016-00000064896-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/73e41be7c4a0/cureus-0016-00000064896-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae72/11330583/9bfa12d0b7af/cureus-0016-00000064896-i07.jpg

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Extraskeletal Ewing sarcoma: Diagnosis, management and prognosis.骨外尤文肉瘤:诊断、治疗与预后
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