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血液透析相关获得性囊性肾病中发生的肉瘤样肾细胞癌伴弥漫性骨髓浸润。

Sarcomatoid renal cell carcinoma arising in hemodialysis-associated acquired cystic kidney disease presenting with disseminated bone marrow infiltration.

作者信息

Suvarna S K, Ahuja M, Brown C B

机构信息

Department of Histopathology, Northern General Hospital, Sheffield, UK.

出版信息

Am J Kidney Dis. 1994 Oct;24(4):581-5. doi: 10.1016/s0272-6386(12)80216-2.

Abstract

A 51-year-old man with a 23-year history of hemodialysis-dependant end-stage renal failure presented with pyrexia, malaise, and progressive pancytopenia. Investigations revealed acquired cystic kidney disease, with one cyst being interpreted as showing evidence of recent hemorrhage, and a malignant sarcomatoid bone marrow infiltrate. The patient rapidly deteriorated and died. At autopsy a seminecrotic and cystic sarcomatoid renal cell carcinoma was identified, with metastases in the adrenals, bone marrow, liver, lungs, and abdominal lymph nodes. The case is reviewed with regard to epithelial membrane antigen- and cytokeratin-negative bone marrow immunohistology as well as the immunophenotype of acquired cystic kidney disease and that expected in renal carcinoma, with reference to the literature on acquired cystic kidney disease.

摘要

一名有23年依赖血液透析的终末期肾衰竭病史的51岁男性,出现发热、不适和进行性全血细胞减少。检查发现患有获得性囊性肾病,其中一个囊肿被认为有近期出血的迹象,且存在恶性肉瘤样骨髓浸润。患者病情迅速恶化并死亡。尸检时发现一个半坏死性和囊性的肉瘤样肾细胞癌,伴有肾上腺、骨髓、肝脏、肺和腹部淋巴结转移。结合上皮膜抗原和细胞角蛋白阴性的骨髓免疫组织学以及获得性囊性肾病和肾癌预期的免疫表型,参考有关获得性囊性肾病的文献对该病例进行了回顾。

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