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原发性肾脏滑膜肉瘤病例系列:罕见实体的临床特征和治疗管理。

Primary Renal Synovial Sarcoma Case Series: Clinical Profile and Management of a Rare Entity.

机构信息

Department of Surgical Oncology, Cancer Institute (WIA), Chennai, India.

Department of Surgical Oncology, Nizam's Institute of Medical Sciences, Hyderabad, India.

出版信息

Urol Int. 2022;106(4):426-428. doi: 10.1159/000520223. Epub 2021 Nov 29.

Abstract

Synovial sarcomas occur predominantly in the extremities. Primary renal synovial sarcoma is a rare entity. Very few cases have been reported in the literature. Clinical and radiological features are similar to renal cell carcinoma with the diagnosis being established after surgery based on histopathology, immunohistochemistry, and chromosome studies. There are no established guidelines on the role of adjuvant treatment in the management of this disease. We herein present a series of 3 cases managed at 2 institutions. In the current series, all patients had venous thrombus, and surgery was the mainstay of treatment. One patient received neoadjuvant chemotherapy after a preoperative biopsy which was done as she did not respond to chemotherapy for a presumptive diagnosis of Wilm's tumor.

摘要

滑膜肉瘤主要发生于四肢。原发性肾脏滑膜肉瘤较为罕见,文献中报道的病例很少。其临床表现和影像学特征与肾细胞癌相似,术后基于组织病理学、免疫组织化学和染色体研究来确诊。目前,对于该疾病的辅助治疗作用尚未有明确的指南。本文报告了在 2 家医疗机构治疗的 3 例病例。在本系列中,所有患者均存在静脉血栓,手术是主要的治疗手段。1 例患者在术前活检后接受了新辅助化疗,因为她对疑似 Wilm 瘤的化疗没有反应。

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