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成人患者中形态学上酷似透明细胞-乳头状肾细胞癌的Xp11易位性肾细胞癌:1例报告,拓展了Xp11易位性肾细胞癌的形态学谱。

Xp11 translocation renal cell carcinoma morphologically mimicking clear cell-papillary renal cell carcinoma in an adult patient: report of a case expanding the morphologic spectrum of Xp11 translocation renal cell carcinomas.

作者信息

Parihar Asmita, Tickoo Satish K, Kumar Sunil, Arora Vinod Kumar

机构信息

University College of Medical Sciences, Delhi, India.

Memorial Sloan-Kettering Cancer, New York, NY, USA.

出版信息

Int J Surg Pathol. 2015 May;23(3):234-7. doi: 10.1177/1066896914562280. Epub 2014 Dec 17.

DOI:10.1177/1066896914562280
PMID:25519951
Abstract

Xp11 translocation renal cell carcinoma (RCC) is a relatively rare tumor mainly affecting children and adolescents. It shows significant morphological overlap with the 2 most common adult renal tumors, which are the clear cell (conventional) RCC and papillary RCC. We describe case of a young adult female who presented with right flank pain and abdominal mass. Radiological investigations showed features suggestive of renal cell carcinoma in the right kidney. Histopathological findings while suggestive of Xp11 carcinoma, showed significant overlap with the recently described entity clear cell papillary RCC. TFE3 immunohistochemistry confirmed the tumor to be Xp11 translocation RCC. The patient had an aggressive course with lymph node metastasis. In this report, we discuss differential diagnosis and the diagnostic challenges of Xp11 translocation RCC in adults.

摘要

Xp11易位性肾细胞癌(RCC)是一种相对罕见的肿瘤,主要影响儿童和青少年。它与两种最常见的成人肾肿瘤——透明细胞(传统型)RCC和乳头状RCC——在形态学上有显著重叠。我们描述了一例年轻成年女性病例,该患者表现为右侧胁腹疼痛和腹部肿块。影像学检查显示右侧肾脏有提示肾细胞癌的特征。组织病理学检查结果虽提示为Xp11癌,但与最近描述的实体——透明细胞乳头状RCC有显著重叠。TFE3免疫组化证实该肿瘤为Xp11易位性RCC。该患者病程进展迅速,出现了淋巴结转移。在本报告中,我们讨论了成人Xp11易位性RCC的鉴别诊断及诊断挑战。

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