本帖最后由 pathology 于 2013-1-29 13:50 编辑
本周病例（2013.1.7—2013.1.13）
1、你考虑什么诊断？
透明细胞管状**状肾细胞癌（Clear Cell Tubulopapillary Renal Cell Carcinoma）
This is a clear cell neoplasm which has prominent smooth muscle in its septa. These cells have low nuclear grade, and have an acinar and papillary architecture. Characteristically, the nuclei are oriented away from the basement membrane, such that there are subnuclear vacuoles in many of the cells. The cells label for cytokeratin 7 and carbonic anhydrase 9, supporting the diagnosis of clear cell tubulopapillary RCC.
CAIX
CK7
Clear cell carcinoma lacks the characteristic subnuclear vacuoles of the current lesion and does not typically label diffusely for cytokeratin 7.
Papillary renal cell carcinoma is usually only focally clear, and does not label diffusely for carbonic anhydrase 9.
Xp11 translocation renal cell carcinomas lack the characteristic subnuclear vacuoles of the current lesion, frequently affect young patients, and are associated with prominent psammoma body formation in many cases. The latter lesions demonstrated nuclear labeling for TFE3 protein which was not true of the current lesion.
Clear cell tubulopapillary renal cell carcinomas are often associated with end stage renal disease, but may arise in otherwise normal kidneys. These are distinctive lesions which lack the loss of chromosome 3p or trisomies of chromosome 7 and 17 typical of clear and papillary renal cell carcinoma, respectively.
2、需要做什么鉴别诊断？
透明细胞肾细胞癌、**状肾细胞癌、Xp11.2易位/TFE3基因融合相关性肾细胞癌
3、本病预后如何？
目前报道的大宗病例来自AJSP的33例病例，在20例有随访资料的病例中，平均随访27.4月（1~85月），其中19例无病生存，另1例伴有VHDL的病例肾部分切除术后病情稳定存活15月。
Ref：
尊重著作权人合法权益，该附件版权审核中
透明细胞管状乳头状肾细胞癌.pdf
(100.8 KB, 下载次数: 17)
尊重著作权人合法权益，该附件版权审核中
Clear Cell Tubulopapillary Renal Cell Carcinoma.pdf
(1.48 MB, 下载次数: 7)
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本期病例非常典型，作为新近报道的一种特殊亚型的肾细胞癌，结合临床特征、组织学形态，结合恰当的免疫组化不难诊断。综合回答思路，rewe、派瑞松加一分！
希望大家一如既往的支持病理每周一题， | 
发表于 2013-1-7 18:34
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