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斯陶尔夫综合征,临床意义及知识缺口,大小重要吗?病例报告。

Stauffer syndrome, clinical implications and knowledge gaps, does size matter? Case report.

机构信息

Division of Urologic Oncology, Pontificia Universidad Javeriana, Hospital Universitario San Ignacio, Carrera 7 No. 40 - 62, Bogotá, Colombia, 11011.

出版信息

BMC Urol. 2020 Jul 20;20(1):105. doi: 10.1186/s12894-020-00671-w.

Abstract

BACKGROUND

Stauffer Syndrome (SS) is a paraneoplastic disorder associated with renal cell carcinoma (RCC). First described by Herbert Maurice Stauffer in 1961, it is characterized by hepatic dysfunction in the absence of metastasis, and elevated alkaline phosphatase, aminotransferases and prolonged prothrombin time. Initial reports did not include jaundice as an inclusion criterion. We aim to report the rare SS jaundice variant in a patient with a small renal mass and review the literature to determine if the size of the renal mass could influence the development of the SS.

CASE PRESENTATION

The aim of this article is to present the case of a 53-year-old male with Stauffer syndrome jaundice variant secondary to a 2.5 cm renal mass, treated with laparoscopic radical nephrectomy with complete resolution of the syndrome.

CONCLUSIONS

This syndrome is yet to be fully understood, and as far as the evidence shows, size does not matter. This entity should always be in mind when encountered with a patient with liver dysfunction and jaundice with a suspicion or confirmed diagnosis of a renal mass. To date, and to our knowledge, there have been eleven reported cases of paraneoplastic cholestatic jaundice syndrome including the current case. Six cases presented in patients with small renal mases (< 4 cm), curiously there were not a laterality tendency.

摘要

背景

Stauffer 综合征(SS)是一种与肾细胞癌(RCC)相关的副肿瘤性疾病。它由 Herbert Maurice Stauffer 于 1961 年首次描述,其特征为无转移的肝功能障碍,碱性磷酸酶、氨基转移酶升高和凝血酶原时间延长。最初的报告并未将黄疸作为纳入标准。我们旨在报告一例伴有小肾肿块的罕见 SS 黄疸变异病例,并复习文献以确定肾肿块的大小是否会影响 SS 的发生。

病例介绍

本文的目的是介绍一例 53 岁男性患者,因 2.5cm 肾肿块继发 Stauffer 综合征黄疸变异,接受腹腔镜根治性肾切除术,该综合征完全缓解。

结论

该综合征尚未被充分理解,就现有证据来看,大小并不重要。当遇到肝功能障碍和黄疸的患者,伴有或确诊为肾肿块时,应始终考虑到这种情况。迄今为止,据我们所知,包括本病例在内,已有 11 例报道的副肿瘤性胆汁淤积性黄疸综合征。6 例患者的肾肿块较小(<4cm),有趣的是,其并无侧别倾向。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6c83/7370459/c699e1faa4e1/12894_2020_671_Fig1_HTML.jpg

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