Primary Renal Synovial Sarcoma Presenting as Haemorrhagic Shock: A Rare Presentation.

IF 1.9 Q3 ONCOLOGY Journal of Kidney Cancer and VHL Pub Date : 2021-09-03 eCollection Date: 2021-01-01 DOI:10.15586/jkcvhl.v8i3.187
Ibrahim Alzahrani, Nasser Albqami, Abdullah Alkhayal, Nourah AlOudah, Ali Alyami
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引用次数: 1

Abstract

Primary synovial sarcoma (PSS) of the kidney is considered the rarest type of all renal sarcomas with specific chromosomal translocation t (X; 18) (p11.2; q11.2). We report the case of a 65-year-old man with no medical conditions who presented to the emergency department with sudden severe right flank pain associated with haemodynamic instability and haemorrhagic shock. Computed tomography (CT) of the abdomen and pelvis revealed a right renal mass. A right open radical nephrectomy was performed. Histopathology revealed a monophasic synovial sarcoma. The patient received six cycles of docetaxel and gemcitabine as adjuvant chemotherapy. No sign of recurrence was seen on a follow-up CT urogram. This rare tumour often presents atypically, and clear guidelines regarding appropriate treatment are lacking. Our case showed that treatment with docetaxel/gemcitabine after an open radical nephrectomy is promising.

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原发性肾滑膜肉瘤表现为出血性休克:一种罕见的表现。
原发性肾滑膜肉瘤(PSS)被认为是所有肾肉瘤中最罕见的类型,具有特异性染色体易位t (X;18) (p11.2;q11.2)。我们报告的情况下,65岁的男子没有医疗条件谁提出了急诊科突然严重的右侧疼痛与血流动力学不稳定和出血性休克。腹部及骨盆电脑断层扫描显示右侧肾脏肿块。行右侧开放性根治性肾切除术。组织病理学显示为单相滑膜肉瘤。患者接受了6个周期的多西他赛和吉西他滨辅助化疗。随访CT尿路图未见复发迹象。这种罕见的肿瘤通常表现为非典型,缺乏关于适当治疗的明确指南。我们的病例表明,开放性根治性肾切除术后多西他赛/吉西他滨治疗是有希望的。
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