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Kidney tumor - adult malignancies

Adult renal cell carcinoma

Papillary type, renal cell carcinoma


Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 24 April 2014, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

See also Type 1, Type 2, solid types below

General
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● Papillary or tubulopapillary tumor with foamy macrophages and intracellular hemosiderin
● Size greater than 5 mm distinguishes tumor from papillary adenoma; frequently trisomy 7, 17
● Also called chromophil; some cases were formerly called renal cell carcinoma-granular type due to granular cytoplasm, but this term is obsolete and may also refer to oncocytoma or eosinophilic variant of clear cell carcinoma
● 10-20% of adult renal carcinomas
Origin: proximal or distal convoluted tubule
Epidemiology: 75% male

Clinical description
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● Tends to present at early stage
● 5 year survival is 82-90%, may be better than clear cell carcinoma (Am J Surg Pathol 2002;26:281)
● Metastasizes to regional lymph nodes

Case reports
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● 63 year old man with bilateral renal masses (Univ of Oklahoma)
● 74 year old man with tumor arising in calyceal cyst (Arch Pathol Lab Med 1996;120:879)
● Focal mucin production (Am J Surg Pathol 1998;22:1037)

Gross description
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● Thick capsule with reactive changes and hemorrhage, red/brown (from hemorrhage); multifocal (80% of tumors), occasionally bilateral; mean 7 cm
● Tissue “pours out" of kidney
● Looks necrotic but microscopically less necrosis than expected

Gross images
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Tan-pink mass with satellite nodules


Golden yellow tumor


Tumors are predominantly cysts with a localized mural nodule in each


Renal vein extension

Large cystic tumor infiltrates perirenal fat

Hemorrhagic and partially necrotic tumor

Multiple tumor nodules, one is hemorrhagic-cystic

Capsular invasion

Micro description
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● Well-circumscribed, often with distinct fibrous capsule
● Papillary or tubulopapillary in every case
● Have papillary fibrovascular cores that may be edematous and look cystic; papillae may be long and solidly packed
● Foamy macrophages in papillary cores and intracellular hemosiderin are sensitive/specific features
● Papillae are composed of columnar/cuboidal cells with finely granular cytoplasm, lower grade nuclei, longitudinal nuclear grooves in low grade cases; often tubular dysplasia
● May have glassy hyaline globules
● Variable psammoma bodies, neutrophils and necrosis
● Focally clear cell areas may be present (J Urol 2011;185:30, Am J Surg Pathol 2008;32:1780)
● One study recommends assessing nucleolar prominence based upon high power field with greatest nuclear pleomorphism (Am J Surg Pathol 2006;30:1091)

Micro images
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Papillary pattern:

Prominent papillary structures with clusters of macrophages

   
Papillary tumors with fibrovascular cores

       
Papillary tumor in cyst wall

               
Oncocytic variant of papillary renal cell carcinoma, contributed by: Dr. Semir Vranic, University of Sarajevo (Bosnia)


A single layer of cuboidal cells covers a fibrovascular papillary stalk


Tumor without macrophages

Tumor cell features:

Tumor cells have eosinophilic cytoplasm and at most mild atypia

Finely granular cytoplasm


Prominent nucleoli


Nuclear anaplasia


Nuclear grooves (fig 4A-D)

   
Papillary structures have numerous stromal xanthoma cells


Cholesterol clefts


Abundant hemosiderin granules


Papillary adenoma versus papillary carcinoma

Cytology description
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● Foamy macrophages and intracytoplasmic hemosiderin (Cancer 1998;84:303)

Cytology images
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Various images

Various images

Positive stains
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Most common:
● AE1-AE3, low molecular weight cytokeratin (CK7) (83% type 1, 20% type 2), CK 8/18, CK19, AMACR (Am J Surg Pathol 2004;28:69, Hum Pathol 2006;37:698)
● EMA/MUC1 (membranous), RCC-Ma, CD10
● Also CD117/c-kit (cytoplasmic, Mod Pathol 2004;17:611)
● Variable vimentin, variable glycogen

Suggested positive staining panel:
● CK7, CK8/CK18, CK19, vimentin (Am J Surg Pathol 2005;29:747)
● AMACR (Am J Surg Pathol 2008;32:1780)

Negative stains
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● 34betaE12 (high molecular weight cytokeratin), Ulex europaeus, parvalbumin, WT1

Electron microscopy description
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● Variably sized microvilli, small amounts of cytoplasmic lipid, no glycogen

Molecular/cytogenetics description
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● +7 (75%), +17 (80%), +12, +16, +20, +3q, -Y, -X (p or q for all chromosomes)
● These changes occur early in tumor neoplasia (Mod Pathol 2003;16:1053), point mutation in c-kit intron 17
● Mutations in MET proto-oncogene on #7 are common
● Also associated with papillary RCC gene on #1
● Not associated with p53 mutations or 3p-

Differential diagnosis
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Papillary adenoma: similar morphology (with or without fibrous capsule) but size 5mm or less by WHO criteria
Collecting duct carcinoma: infiltrates tubules, desmoplasia
Clear cell carcinoma with focal papillary architecture: dyshesive tumor areas may have pseudopapillary appearance – are AMACR-, CK7-; 3p deletion present, no +7 or +17, no Y-
● Papillary carcinoma with focal clear cell areas: clear cell areas show morphology similar to foamy macrophages within papillae – AMACR+, CK7+; +7 or +17, Y-, usually no 3p deletion (Am J Surg Pathol 2008;32:1780)
Translocation carcinoma: TFE3 positive by immunohistochemistry, often papillary architecture with intermingled areas of both clear and eosinophilic cells, limited keratin reactivity (Am J Surg Pathol 2010;34:1295)

Additional references
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Hum Pathol 2001;32:590 (type 1 vs. 2), Am J Surg Pathol 1997;21:621


Type 1 (basophilic) papillary renal cell carcinoma

General
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● More common and better prognosis than type 2

Micro description
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● Cells arranged on single layer on basement membrane of papillary cores
● Small cells with scant pale cytoplasm and hyperchromatic nuclei so cell looks blue
● Frequent psammoma bodies, foamy macrophages, glomeruloid papillae; lower nuclear grade than type 2

Micro images
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Thin papillae covered by small basophilic cells with round nuclei


Small basophilic tumor cells (fig A)

Small basophilic tumor cells and foam cells


Diffuse apical MUC1 staining

Positive stains
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● CK7, MUC1 (polarized expression, BJU Int 2008;102:183)

Molecular description
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● Associated with c-met mutation


Type 2 (eosinophilic) papillary renal cell carcinoma

General
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● Poorer prognosis than type 1; typically presents at higher stage

Gross description
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● Often distinct fibrous capsule

Gross images
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Irregular friable yellow tumor of the upper pole

Large cystic tumor

Micro description
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● Papillae covered by larger cells with abundant eosinophilic granular cytoplasm and grade 3 nuclei (prominent nucleoli), pseudostratified nuclei, variable foam cells and necrosis

Micro images
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Tumor papillae covered by large eosinophilic cells with irregular nuclei


Fig. B


Rare and focal MUC1 expression (arrows)

Cytology description
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● Papillary structures, foamy histiocytes, intracytoplasmic hemosiderin and nuclear grooves

Positive stains
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● Hale colloidal iron due to hemosiderin
● Also topoisomerase II alpha (Cancer Res 2005;65:5628)

Negative stains
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● MUC1

Molecular description
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● 1p-, 3p-, +5q (Clin Cancer Res 2009;15:1162)

Electron microscopy description
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● Similar to classic type but with more mitochondria


Solid variant of papillary renal cell carcinoma

Micro description
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● Solid sheets of cells, often with distinct micronodules resembling abortive papillae
● Less than 50% true papillae but otherwise similar to classic papillary (Am J Surg Pathol 1997;21:1203)
● Cells have abundant eosinophilic cytoplasm, open chromatin, often prominent nucleoli
● May have clear cytoplasm and nuclear grooves
● May have compact areas of low grade spindle cells lining thin, angulated tubules resembling mucinous tubular and spindle cell carcinoma, but no mucinous stroma (Am J Surg Pathol 2008;32:1353)

Positive stains
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● EMA, CK7

Negative stains
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● 34betaE12

Molecular description
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● Trisomy 7 and 17

Differential diagnosis
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Mucinous tubular and spindle cell carcinoma: spindle cell areas are uniformly low grade, no mucinous stroma, no +7 or +17

End of Kidney tumor - adult malignancies > Adult renal cell carcinoma > Papillary type, renal cell carcinoma


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