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原发性肾尤文肉瘤:一例报告并文献复习

A primary Ewing's sarcoma of the kidney: A case report and review of literature.

作者信息

Senkhum Ornsinee, Attawettayanon Worapat, Pripatnanont Choosak, Chalieopanyarwong Virote

机构信息

Department of Surgery, Division of Urology, Faculty of Medicine, Songklanagarind Hospital, Prince of Songkla University, Hat Yai, Songkhla, Thailand.

出版信息

Urol Ann. 2023 Jul-Sep;15(3):334-336. doi: 10.4103/ua.ua_2_23. Epub 2023 Jul 17.

Abstract

Primary Ewing's kidney sarcoma is a rare and aggressive cancer with poor treatment outcomes. Furthermore, clinical presentations are nonspecific, such as abdominal pain, abdominal mass, cachexia, or hematuria. Currently, there is no consensus guideline for the treatment of this condition. We report on a 22-year-old man who presented with gross hematuria for 3 weeks. Computed tomography demonstrated a huge right renal mass with a thrombus in the inferior vena cava (IVC) without visceral metastasis. A percutaneous tissue biopsy revealed Ewing's sarcoma (ES) and the patient received neoadjuvant chemotherapy (doxorubicin and ifosfamide) for four cycles. This reduced the tumor's size significantly and made it eligible for surgical intervention. Radical nephrectomy and tumor thrombus removal with wedge IVC wall were performed. The final diagnosis of ES was confirmed by the immunohistochemistry of the kidney specimen. After a 5-year follow-up of the patient, clinical and imaging evidence demonstrated that there was no disease recurrence.

摘要

原发性尤因肾肉瘤是一种罕见且侵袭性强的癌症,治疗效果不佳。此外,临床表现不具特异性,如腹痛、腹部肿块、恶病质或血尿。目前,对于这种疾病的治疗尚无共识性指南。我们报告一例22岁男性,出现肉眼血尿3周。计算机断层扫描显示右肾有巨大肿块,下腔静脉有血栓形成,无内脏转移。经皮组织活检显示为尤因肉瘤(ES),患者接受了四个周期的新辅助化疗(多柔比星和异环磷酰胺)。这显著缩小了肿瘤大小,使其符合手术干预条件。进行了根治性肾切除术及下腔静脉壁楔形肿瘤血栓切除术。肾标本的免疫组织化学检查确诊为ES。对该患者进行5年随访后,临床和影像学证据表明无疾病复发。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/96c6/10471821/daba061b2f2a/UA-15-334-g001.jpg

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