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Kidney tumor - adult malignancies

Adult renal cell carcinoma

Clear cell papillary renal cell carcinoma


Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 16 May 2014, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Tumor cells with clear cytoplasm and papillary architecture, lacking genetic changes of classic papillary carcinoma (no +7 or –Y), and classic clear cell carcinoma (no 3p-)
● Generally Fuhrman grade 1-2 without oxalate crystals (in contrast to acquired cystic disease related carcinomas), may include cases otherwise unclassified (Am J Surg Pathol 2008;32:1780), also called clear cell tubulopapillary renal cell carcinoma (Am J Surg Pathol 2010;34:1608)

Epidemiology
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● Often associated with end stage renal disease (Am J Surg Pathol 2006;30:141), but may occur in otherwise normal kidneys (Am J Surg Pathol 2008;32:1239)

Clinical description
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● Good prognosis, with no recurrence or metastases

Micro description
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● May have areas with striking resemblance to typical clear cell RCC
● Tumor cells have clear cytoplasm and papillary architecture (often focal)
● Frequently a cystic component
● Cells have nuclei aligned away from the basement membrane (resembling secretory pattern endometrium)
● Generally Fuhrman grade 1-2, no oxalate crystals

Micro images
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Clear cell tumors with papillary architecture

Contributed by Dr. Sean Williamson:
                       

Positive stains
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● AE1/AE3, CK7, CAM 5.2, Vimentin; also EMA, carbonic anhydrase IX (Int J Surg Pathol 2012;20:19)

Negative stains
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● AMACR, CD10, RCC, TFE3; low PCNA (Mod Pathol 1998;11:339, Hum Pathol 2002;33:230)

Molecular description
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● Non-classic molecular mechanisms – appears to have activation of hypoxia-inducible factor pathway without typical VHL mechanism (Mod Pathol 1999;12:301, Arch Pathol Lab Med 1986;110:592, Mod Pathol 2011;24:1207)
● No characteristic genomic imbalances (Histopathology 2011;58:1064)

Differential diagnosis
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Clear cell renal cell carcinoma: may have a component of pseudo–papillary architecture, particularly in high grade tumors; CK7-, AMACR-, has 3p-, no +7, no +17, no -Y (Arch Pathol Lab Med 2003;127:1176)
Papillary renal cell carcinoma: clear cell areas typically are focal; AMACR+, CK7+, either +7 or +17, -Y, usually no 3p-
Multilocular cystic renal cell carcinoma: both tumors may have a prominent cystic component but multilocular cystic RCC should have no large, expansile solid areas or papillary structures
Xp11 translocation carcinoma: often a mixture of eosinophilic and clear cells, TFE3+

End of Kidney tumor - adult malignancies > Adult renal cell carcinoma > Clear cell papillary renal cell carcinoma


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