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Managing Renal Cell Carcinoma Associated Paraneoplastic Syndrome with Nephron-sparing Surgery in a Patient with von Hippel-Lindau

机译:von Hippel-Lindau患者的肾细胞癌伴副肿瘤综合征伴保肾手术

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摘要

A patient with germline von Hippel-Lindau (VHL) gene alteration and history of multiple tumors present with classical paraneoplastic syndrome (PNS) associated with renal cell carcinoma (RCC). She underwent open nephron sparing surgery with resolution of symptoms. She remained without recurrence of RCC for the initial 2?years of her follow-up. To the best of our knowledge, this case represents the first in which PNS was specifically resolved using a partial nephrectomy in a patient with VHL. This case report provides initial evidence for the potential role of nephron sparing surgery in the management of paraneoplastic symptoms associated with hereditary RCC.
机译:患有生殖细胞冯·希佩尔·林道(VHL)基因改变和多发性肿瘤并伴有肾癌(RCC)的经典副肿瘤综合征(PNS)病史的患者。她接受了保留肾气的开放性手术,以缓解症状。在随访的最初2年中,她仍然没有RCC复发。据我们所知,该病例代表了首例使用部分肾切除术对VHL患者进行PNS特异性治疗的病例。该病例报告提供了备用肾单位手术在遗传性RCC相关副肿瘤症状管理中的潜在作用的初步证据。

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