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局限性原发性输尿管淀粉样变性

Localized primary ureteral amyloidosis.

作者信息

García-Escudero López A, Padilla Nieva J, Infante Riaño R, Martín Bazaco J, Arruza Echevarría A, Moreno Nieto V

机构信息

Urology Department, Hospital Txagorritxu, Vitoria, Alava, Spain.

出版信息

Arch Esp Urol. 2011 May;64(4):371-5.

Abstract

OBJECTIVE

To analyze a new case of primary localized amyloidosis of the ureter, describing the differential diagnosis with ureteral tumors and its pathogenic mechanism.

METHOD

Description of the case of a male patient who presented clinical symptoms of renoureteral pain and hematuria and an image suspected of ureteral tumour undergoing laparoscopic nephroureterectomy. The pathological study confirmed amyloidosis of the ureter, AL by immunohistochemistry.

RESULTS

This report further extends the slightly more than 50 published cases of primary localized amyloidosis of the ureter the clinical signs of which, pain and haematuria, are similar, showing stenosis and enlargement in an area of the ureteral wall on imaging techniques. In the majority of cases the diagnosis was obtained postoperatively, being necessary to exclude generalized and secondary affectation.

CONCLUSIONS

Emphasis must be placed on the differential diagnosis of tumors of the ureter, especially in single-kidney patients, by using ureteroscopy and biopsy or preferably a careful cytological study. In our case a local inflammatory phenomenon could have been the basis of its pathogenesis.

摘要

目的

分析一例输尿管原发性局限性淀粉样变性新病例,描述其与输尿管肿瘤的鉴别诊断及其发病机制。

方法

描述一名男性患者的病例,该患者出现肾输尿管疼痛和血尿的临床症状,影像学检查怀疑为输尿管肿瘤,遂接受腹腔镜肾输尿管切除术。病理研究证实为输尿管淀粉样变性,免疫组化显示为AL型。

结果

本报告进一步扩展了已发表的略多于50例的输尿管原发性局限性淀粉样变性病例,其临床症状疼痛和血尿相似,影像学检查显示输尿管壁某区域有狭窄和扩大。大多数病例在术后确诊,有必要排除全身性和继发性病变。

结论

必须重视输尿管肿瘤的鉴别诊断,尤其是对单肾患者,可采用输尿管镜检查和活检,或最好进行仔细的细胞学研究。在我们的病例中,局部炎症现象可能是其发病机制的基础。

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