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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: 30 June 2012, 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chromophil; some cases were formerly called renal cell carcinoma-granular type due to granular cytoplasm, but this term is obsolete and may also refer to oncocytoma or eosinophilic variant of clear cell carcinoma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
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
Large cells with abundant eosinophilic cytoplasm and prominent nucleoli
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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