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病例报告:表现为温德利希综合征的原发性肾肉瘤行部分肾切除术;一种罕见肿瘤的罕见表现,采用非典型治疗。

Case Report: Partial nephrectomy in primary renal sarcoma presenting as Wunderlich syndrome; a rare tumour with rare presentation managed atypically.

作者信息

Manikandan Ramanitharan, Mehra Ketan, Dorairajan Lalgudi Narayanan, Nachiappa Ganesh Rajesh, Sreenivasan Sreerag K, Kumar Rajeev

机构信息

Department of Urology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, 605006, India.

Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, 605006, India.

出版信息

F1000Res. 2019 Apr 10;8:423. doi: 10.12688/f1000research.18698.1. eCollection 2019.

Abstract

Spontaneous retroperitoneal haemorrhage also called Wunderlich Syndrome (WS) may be caused by various aetiologies. One of the most common causes is renal tumour. Renal sarcoma is a rare cause of WS, and renal sarcoma in itself is a rare entity. In the era of nephron-sparing surgery, optimum management of primary renal sarcoma remains a dilemma as there are limited number of cases available in the literature. Nevertheless, radical nephrectomy remains the recommended treatment, keeping in mind the aggressiveness of the tumour. We report a case of primary undifferentiated renal sarcoma, which presented as WS, and which was managed by partial nephrectomy.

摘要

自发性腹膜后出血也称为温德利希综合征(WS),可能由多种病因引起。最常见的病因之一是肾肿瘤。肾肉瘤是WS的罕见病因,而肾肉瘤本身就是一种罕见的疾病。在保留肾单位手术的时代,原发性肾肉瘤的最佳治疗方案仍然是一个难题,因为文献中报道的病例数量有限。尽管如此,考虑到肿瘤的侵袭性,根治性肾切除术仍然是推荐的治疗方法。我们报告一例原发性未分化肾肉瘤,表现为WS,采用部分肾切除术进行治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3c50/6652101/1ecdb597527a/f1000research-8-20474-g0000.jpg

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