Kidney tumours

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Kidney tumours - includes malignant kidney tumours and benign kidney tumours. Medical renal diseases are dealt with in the medical renal diseases article.

Pediatric kidney tumours are dealt with in the pediatric kidney tumours article.

Renal specimens

  • Renal biopsy - usually for renal onocytoma vs. renal cell carcinoma (RCC) or medical diseases - see medical kidney.
  • Partial nephrectomy.
  • Nephrectomy.
  • Nephroureterectomy (includes ureter) - done for urothelial cell carcinoma (UCC) of the renal pelvis and ureteric UCC.
  • Radical nephrectomy - includes Gerota's fascia.

In excisions done for tumours, a comment should be made about kidney distant from the tumour. People with less renal mass, i.e. less kidney, are predisposed to focal segmental glomerulosclerosis (FSGS).

Anatomy

The anatomy is important for properly staging renal neoplasms.

Layers (superficial to deep):

  1. Renal fascia (Gerota's fascia).
  2. Perinephric fat.
  3. Renal capsule.
  4. Renal parenchyma (cortex).

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Missed renal biopsy

KIDNEY (LESION), LEFT, CORE BIOPSY:
- RENAL PARENCHYMA.
- NEGATIVE FOR MASS LESION, SEE COMMENT.

COMMENT:
No mass lesion is apparent in the tissue sampled. A re-biopsy should be considered.

Renal parenchyma:
- Glomeruli: seven glomeruli sampled, no apparent glomerular pathology on the H&E sections.
- Interstitium: interstitial fibrosis is not identified.
- Tubules: no pathology is apparent.
- Vessels: mild atherosclerosis, no hyalinization of arterioles apparent.

Tabular comparison (selected tumours)

Selected common tumours of the kidney:[2][3]

Clear cell RCC Papillary RCC
type 1 		Papillary RCC
type 2 		Chromophobe RCC
classic variant 		Chromophobe RCC
eosinophilic variant 		Oncocytoma
Gross Golden yellow, solid friable friable light brown light brown mahogany/brown, +/-central scar
Architecture nests, sheets papillary, simple papillary,
pseudostratified 		nests, sheets nests, sheets nests, sheets
Nuclear atypia low-high
typically medium-high 		low-medium medium-high low-high, "raisinoid"
nuc. membrane 		low-high, "raisinoid"
nuc. membrane 		low-medium, round nuclei
Cytoplasm clear eosinophilic eosinophilic cobwebs/clear eosinophilic/cobwebs eosinophilic/
granular & abundant
Other delicate vessels,
necrosis common 		histiocytes
in fibrovascular cores, >0.5 cm 		histiocytes
in fibrovascular cores, >0.5 cm 		perinuclear clearing, thick vessels perinuclear clearing, thick vessels in loose fibrous/hyaline stroma
IHC CK7-, EMA+ AMACR+, EMA+, CK7+ AMACR+, E-cadherin+, CK7- CD117+, CK7+ (membrane) CD117+, CK7+ (membrane) Vimentin-, EMA+
Main DDx chromophobe
classic variant 		PaRCC type 2, mets PaRCC type 1, mets clear cell RCC oncocytoma chromophobe
eosinophilic variant
Key features clear cells, vascular papillae, histiocytes
simple epithelium 		papillae, histiocytes,
stratified 		perinuc. clearing,
wispy cytoplasm 		perinuc. clearing,
wispy eosinophilic
cytoplasm 		eosinophilic, granular cytoplasm
Image(s)
 
CCRCC (WC)
 
PaRCC - intermed. (WC)
,
 
PaRCC - high (WC)
 
ChRCC (WC)
 
Oncocytoma (WC)

Notes:

  • Cell shape: all have epithelioid morphology.

Tabular comparison of oncocytoma and chromophobe RCC

Histomorphologic features useful to distinguish chromophobe RCC (eosinophilic variant) and oncocytoma:[4]

Morphologic feature ChRCC (eosinophilic
variant) 		Renal oncocytoma
Nuclear morphology "raisinoid"/wrinkled appearance round with small nucleolus,
usu. little size variation
Multinucleation common - binucleation uncommon
Chromatin coarse fine
Architecture solid, crowded nests spaced nests /
archipelago-like, solid
Cytoplasm perinuclear halo, may be focal no perinuclear halo
Degenerative foci
(focal atypia & pleomorphism) 		absent present in ~20% of cases
Image
 
ChRCC (WC/Nephron)
 
Oncocytoma (WC/Nephron)

Common DDx

Spindle cell tumours:

WHO classification

  • Based on 2004 iteration - as per WMSP, slightly modified.[5] Online, the classification can found here.

Renal cell tumours

Common:

Less common:

Metanephric tumours

Nephroblastic tumours

Mesenchymal tumours

Childhood:

Adults:

Other:

Mixed mesenchymal and epithelial tumours

Others

Renal cell carcinoma

Overview

General

  • Relatively common form of cancer.
  • Often abbreviated RCC.
  • AKA hypernephroma.[6]
  • RCC represents approx. 90% of malignancies in kidneys of adults.[7]

Origin

  • Proximal renal tubule.

Clinical

  • Classically described as a triad:[8]
    • Hematuria (most common symptom).
    • Abdominal mass.
    • Flank pain.
  • Frequently picked-up on imaging (incidentaloma) ~ 1/3 of cases.

Risk factors

Subtypes of RCC

RCC (renal cell carcinoma) comes in different subtypes:[9]

  • Clear cell carcinoma (70-80% of RCC) -- abbrev. CCRCC,
  • Papillary renal cell carcinoma (10-15% of RCC) -- abbrev. PRCC,
  • Chromophobe renal carcinoma (5% of RCC) -- abbrev. ChRCC,
  • Collecting duct (Bellini duct) carcinoma (1% of RCC).

Notes:

  • Subtype is an independent predictor of mortality - but adds very little to multivariate models with staging information.[10]
  • CCRCC tends to be worse than ChRCC and PRCC, probably due to higher incidence of mets.[11]
  • The exam answer (worst to best): clear cell RCC, papillary RCC, chromophobe RCC.

IHC - is it RCC?

  • RCC Ma (+), CD10 (+) -- specific for RCC[12]

IHC - differentiation of types

  • Clear cell RCC vs. papillary RCC:
    • CK7 (-ve CCRCC), AMACR (+ve in PRCC).[13]
  • Papillary RCC type 1 vs. papillary RCC type 2:
    • E-cadherin +ve in PRCC type 2.[14]
    • EMA (MUC1) +ve in PRCC type 1.[14]
  • ChRCC vs. oncocytoma (ONC):
    • CK7 (ChRCC +ve membrane), CK20, CD15.[13]
    • CK7 -- ChRCC 86% +ve vs. ONC 0% +ve.[15]
    • CD15 -- ChRCC 11% +ve vs. ONC 57% +ve.[16]
    • Hale's colloidal iron +ve in ChRCC, usually neg. in ONC.[17]
    • PAX2 -- ChRCC (1/11) +ve vs. ONC (20/23) +ve.[18]
    • Kidney-specific cadherin (Ksp-cadherin) -- ChRCC 97% +ve (distinctive membrane pattern) vs. ONC only 3% +ve.[19]
  • ChRCC & renal oncocytoma vs. others:
    • CD117 (ckit) +ve (100% membrane, ~75% cytoplasmic).[20]
  • Clear cell RCC vs. chromophobe RCC:
    • Hale's colloidal iron (+ve in ChRCC).[17]
    • CK7 (cell membrane +ve in ChRCC).

Notes:

  • One paper[21] describes CD10, parvalbumin, AMACR, CK7 and S100A1 as being useful.
  • Another paper I came across:[22]
  • c-kit (CD117) not useful for differentiating ONC and ChRCC.[18]
  • E-cadherin not useful for differentiating ChRCC and ONC.[23]

RCC vs. Urothelial cell carcinoma

Main article: Urothelium
  • Clinically/radiologically, it may not be possible to differentiate renal pelvis UCC and RCC if the tumour is large.
  • Pathologically, this is not very difficult.
  • On gross specimens, it is almost always obvious what one is dealing with:
    • UCC = nephroureterectomy.
    • RCC = partial nephrectomy, nephrectomy or radical nephrectomy.

Renal cell carcinoma with sarcomatoid differentiation

  • AKA sarcomatoid renal cell carcinoma.

General

Features:[24]

  • Not recognized as a distinct entity in 2004 WHO classification.[25]
    • It is considered an indicator of progression.
    • Previously considered a distinct entity.[25][26]
  • Tend to present at higher stage.
  • Worse prognosis when adjusted for stage.
  • Fuhrman grade 4 by definition.

Microscopic

Features:[24]

  1. Renal cell carcinoma.
  2. Sarcomatoid component:

Note:

  • In essence, any kidney tumour with spindle cells[27] or rhabdoid cells should make one think of this.

Images:

Hereditary renal cell carcinoma

The classics - which are all autosomal dominant:[9]

  1. Von Hippel-Lindau syndrome.
    • VHL gene mutation.
    • Clear cell RCC.
  2. Hereditary clear cell renal cell carcinoma.
    • VHL gene mutation.
  3. Hereditary papillary renal cell carcinoma.
    • MET proto-oncogene mutation.
    • PaRCC type 1.[29]
  4. Hereditary leiomyomatosis and renal cell cancer:[29]
  5. Birt–Hogg–Dubé syndrome:[29]
    • FLCN (folliculin) gene mutation.[31]
    • Skin lesions (fibrofolliculoma, trichodiscoma, acrochordon).
    • ChRCC most common, other types seen (e.g. oncocytoma).
    • Variable penetrance (autosomal dominant).

Others:

  • Hereditary papillary carcinoma (TFE3 related translocations).[32]

Notes:

  • A total of ten hereditary renal cancer syndromes have been described. In eight of the ten the gene is known.[33]

Molecular

Recurrent molecular changes in RCC:

  • Clear cell RCC:
    • Loss of 3p - contains the VHL gene.
  • Papillary RCC:
    • Sporadic:
      • Trisomy 7, 16, 17.
      • Loss of Y.
    • Familial:
      • Trisomy 7 - contains MET gene.[34]

Renal cell carcinoma grading

  • AKA Fuhrman grade.

General

Some RCC subtypes are graded based on the Fuhrman system which considers:[35]

  • Nuclear pleomorphism (size, shape).
  • Chromatin pattern.
  • Nucleoli prominence.

Notes:

Criteria & grades

Note:

  • Most tumours are grade 2 & 3.

Fuhrman grading in short

  • 1 vs. 2: grade 2 has granular chromatin, grade 2 has nucleoli visible @ 20x objective.[37]
  • 2 vs. 3: grade 3 has nucleoli @ 10x objective.
  • 3 vs. 4: grade 4 has pleomorphism/hyperchromasia (or spindle cells or rhabdoid cells).

Clear cell renal cell carcinoma

Main article: Clear cell renal cell carcinoma

Multilocular cystic renal cell carcinoma

General

  • No recurrences or metastasis in the literature.[38]
    • This makes one wonder... is it really cancer.
  • Case report rare.[39]

Gross

Features:[38]

  • Cystic with thin septa.
  • Well circumscribed.

Note:

  • This tumour, radiologically, can often be separated from other cystic tumours.[40]

Microscopic

Features:[38]

  • Polygonal cells within the septa.
  • Clear cytoplasm.
  • +/-Calcification (common).

DDx:

  • Cystic renal disease with macrophages in the septa.
  • Cystic clear cell renal cell carcinoma.

IHC

  • EMA +ve.
  • Keratins +ve.
  • CD68 -ve.

Papillary renal cell carcinoma

  • Abbreviated PRCC, PaRCC and papillary RCC.

General

  • Often subclassified[41] into type 1 and type 2 -- see microscopic.
    • Type 1 and Type 2 are different on a cytogenetic and molecular basis.[42]

Epidemiology

Microscopic

Features:[44]

  • Cuboidal or low columnar cell in papillae.
  • Interstitial foam cells in vascular cores - key feature.[45]
    • Most sensitive and specific feature of PRCC.[46]
  • Highly vascular.

Size criterion:

  • Papillary lesions must be >0.5 cm to be called carcinoma; smaller lesions (<=0.5 cm) are called papillary adenomas.[47]

Mnemonic HIP: highly vascular, interstitial foam cells, papillae.

DDx:

Images

  • PaRCC - intermed. mag. (WC/Nephron)

  • PaRCC - high mag. (WC/Nephron)

Histological subtyping

Subtypes:[41]

  • Type 1 - single layer of cells on basement membrane.
    • usually low grade nuclear features, i.e. low Fuhrman grade.
  • Type 2 - pseudostratification of cells.
    • Usually high grade nuclear features, i.e. high Fuhrman grade.

Others:

  • Oncocytic - oncocytic cytoplasm.
    • Extremely rare ~ largest series is 12 cases.[3]

IHC

Features:[41]

  • AMACR +ve.[48]
  • HMWCK (34betaE12) +ve.
  • Panker (AE1/AE3) +ve.
  • CK7 +ve ~90% of type 1, 20% of type 2.

More reading:

Molecular

Features:[9]

  • Sporadic: trisomies 7, 16, 17.
  • Familial: trisomy 7.
    • Chromosome 7 = location of MET gene.

Note:

  • Not used for diagnosis.[49]

Sign out

KIDNEY, RIGHT, NEPHRECTOMY:
- PAPILLARY RENAL CELL CARCINOMA, ONCOCYTIC -- SEE COMMENT;
- FUHRMANN GRADE 2;
- SURGICAL MARGINS NEGATIVE;
- PLEASE SEE TUMOUR SUMMARY.

COMMENT:
The oncocytic variant of papillary renal cell carcinoma (RCC) is uncommon and not widely
recognized as a subtype of papillary RCC.  The prognostic significance of the oncocytic 
cytoplasm is uncertain.[1]  The histomorphology in this case is compatible with a type 1 
papillary RCC.

1. Ann Diagn Pathol. 2006 Jun;10(3):133-9.

Chromophobe renal cell carcinoma

  • Abbreviated ChRCC.

General

  • Least common of the common types of RCC.
  • Fuhrman grading for this entity is controversial, as it does not appear to have any predictive value.[36]

There are two subtypes:[50]

  • Classic.
  • Eosinophilic variant.

Gross

  • Tan, light-brown.
  • Solitary.
  • Well-circumscribed.

Image:

Microscopic

Classic

Features - classic type (3 P's mnemonic):[51][50]

  • Pale cytoplasm, with wisps of eosinophilic material; the cells are not completely clear, they have "cobwebs".
  • Perinuclear clearing, i.e. a pale halo surrounds the nucleus - key feature.
  • Periphery of cell distinct, i.e. cell membrane is easy to discern.

Notes:

  • May have psammoma bodies.
  • May be described as "plant-like"; plant cells have (thick) cell walls.
  • The perinuclear clearing is often somewhat patchy, i.e. it is usually not present in very tumour cell.

DDx:

  • Clear cell RCC (classic).
    • Perinuclear clearing is not seen in clear cell RCC.
    • ChRCC has wisps in the cytoplasm.

Eosinophilic variant

Features - eosinophilic variant:[50]

  • Eosinophilic (finely granular) cytoplasm.
  • Perinuclear clearing - key feature.
  • Periphery of cell distinct.
  • Smaller cells than classic subtype.

Notes:

  1. May have psammoma bodies.

DDx:

  1. Oncocytoma - particularly the eosinophilic variant.
    • IHC may be useful to differentiate (CK7: oncocytoma = cytoplasm +ve, chromophobe = cell membrane +ve).
    • A comparison based on histomorphology: Tabular comparison between ChRCC & oncocytoma.
      • Oncocytoma typically has: no perinuclear clearing, no raisinoid nuclei, no binucleation.
  2. Clear cell RCC, eosinophilic variant.
    • Perinuclear clearing is not seen in clear cell RCC.
    • ChRCC has wisps in the cytoplasm.

Image

  • Oncocytic chromophobe RCC. (WC/Nephron)

www:

Stains

Images:

IHC

  • CK7 +ve cell membrane.[50]
  • CD117 +ve.
  • Vimentin -ve.

Molecular

  • Extensive aneusomy (monosomy?):[38]
    • Loss of chromosomes: 1, 2, 6, 10, 13, 17, 21.

Sign out

KIDNEY, RIGHT UPPER POLE, PARTIAL NEPHRECTOMY:
- CHROMOPHOBE RENAL CELL CARCINOMA.

COMMENT:
The sections show a mix of clear cells with wispy cytoplasm, and cells with eosinophilic
cytoplasm and perinuclear halos. There are no true papillae. 

Stains and immunostains:
Positive: CK7, CAM5.2, EMA, pankeratin, CD117, colloidal iron.
Negative: AMACR, CD10, CD68, RCC, vimentin.

Clear cell papillary renal cell carcinoma

  • AKA clear cell tubulopapillary renal cell carcinoma.[53]
  • Abbreviation CCPRCC.

General

  • New entity not in the WHO classification.

Microscopic

Features:[53]

  • Features of both:
    1. Clear cell RCC.
    2. Papillary RCC.
      • Often do not have true papillae.
  • Apical nuclei - key feature.[54]
    • In most glandular structures the nuclei are usu. basally located, i.e. in the cytoplasm adjacent to the basement membrane.

DDx:

Images

  • CCPRCC - high mag. (WC/Nephron)

  • CCPRCC - very high mag. (WC/Nephron)

www:

IHC

Features:[53]

  • CK7 +ve.
    • Usually -ve in clear cell RCC.
  • CD10 -ve.
    • Usually +ve in clear cell RCC.
    • Positive in Xp11.2 translocation carcinoma.[55]
  • TFE3 -ve.
  • AMACR -ve.
  • Smooth muscle actin +ve (focal).[56]

Others:[53]

  • HIF-1alpha +ve.
  • GLUT-1 +ve.

How to remember: The two key stains are opposite of what the name implies.

  • Clear cell RCC: CK7 negative. In this tumour it is the opposite - CK7 is positive.
  • Papillary RCC: AMACR positive. In this tumour it is the opposite - AMACR is negative.

Unclassified renal cell carcinoma

  • Abbreviated URCC.

General

  • Uncommon.
  • A WHO classification diagnosis of exclusion.
  • Worse prognosis than clear cell renal cell carcinoma.[57]
  • High variation in the prevalence (when comparing institutions); this suggests a lack of uniformity in the diagnosis of this subtype.[57]

Microscopic

Features:

  • Malignant tumour that is one of the following:[58][59]
    1. A combination of other RCC histologic types (~35% of cases of URCC).
    2. Has a "non-identifiable" pattern/unrecognizable cell type (~60% of cases of URCC).
    3. Pure sarcomatoid RCC without an identifiable (epithelioid) RCC subtype (~5% of cases of URCC).[59]

DDx:

Renal translocation carcinomas

Renal tumour with Xp11.2 translocation

General

  • Defined by the presence of a fusion gene formed with TFE3 @ Xp11.2.
  • TFE3 is the gene involved in the translocation seen in alveolar soft part sarcoma (ASPS).
  • Poor prognosis ~ 50% present at stage IV, majority of lymph node metastases.
  • ~1/3 of childhood RCC.[60]

Microscopic

Features:[61]

  • Large cells.
  • Clear or eosinophilic cytoplasm.
  • Papillae or nests.
  • Psammoma bodies - common.[62]
    • Calcification is considered the classic histomorphologic feature.
  • Hyaline bodies - common.

Notes:

DDx:

Images

  • Xp11.2 translocation RCC - intermed. mag. (WC/Nephron)

  • Xp11.2 translocation RCC - high mag. (WC/Nephron)

www:

IHC

  • TFE3 +ve (nucleus) - key feature.[61]
  • CD10 +ve.
  • Vimentin +ve.
  • CK7 -ve.
    • Positive in ~20% of cases.[55]

Others:

Molecular

Renal tumour with t(6;11) translocation

General

Microscopic

Features:

  • Hyaline material between nests.
  • Large cells with clear to eosinophilic cytoplasm.

Molecular

  • t(6;11)(p21;q12) Alpha/TFEB.[64]

Benign tumours

Papillary adenoma of the kidney

  • AKA renal papillary adenoma.

General

Microscopic

Features:

  1. Histomorphology of papillary renal cell carcinoma.
  2. Must be <=0.5 cm.[47][66]

DDx:

Images:

IHC

Renal oncocytoma

General

  • Can be difficult to distinguish radiologically from RCC (chromophobe subtype).
    • ... and pathologists occasionally struggle like the radiologists.
  • Benign tumour - the reason it is excised is... one cannot be certain it isn't a RCC.

Gross

  • Brown, mahogany brown.
  • 1/3 have a characteristic central scar.[67]

Image

  • Renal oncocytoma with central scar. (WP)

Microscopic

Features:

  • Eosinophilic cytoplasm - slightly granular key feature.
  • Cells arranged in nests.
  • Nuclei uniform and round.[67]
    • Slightly enlarged nuclei, but no significant pleomorphism (size variation) - important.

Notes:

  • May look like eosinophilic variant of chromophobe RCC -- this is the main DDx.

DDx:

Images

  • Oncocytoma - high mag. (WC/Nephron)

  • Oncocytoma - intermed. mag. (WC/Nephron)

  • Oncocytoma - low mag. (WC/Nephron)

Stains

IHC

  • CK7 -ve[15]/+ve (cytoplasmic) .
    • Chromophobe renal cell carcinoma = cell membrane +ve.

Sign out

PORTION OF KIDNEY, RIGHT, PARTIAL NEPHRECTOMY:
- ONCOCYTOMA.

Micro

The sections show a tumour with cells arranged in nests. The tumour cells have abundant eosinophilic cytoplasm. The tumour cell nuclei are round and have round nucleoli. No perinuclear halos are apparent. Binucleation is not apparent. No zonal necrosis is identified. Focally, tumour nests are spaced reminiscent of an archipelago. Mitoses are not apparent. The tumour is moderately circumscribed.

The thin rim of renal parenchyma has no apparent pathology on the H&E stained sections.

Angiomyolipoma

  • Abbreviated AML.

General

  • Benign mesenchymal tumour.
  • Presentations: flank pain, hematuria, incidentaloma.[68]
    • Tumours >4 cm considered a risk for bleeding.[69]
  • AMLs occur may be elsewhere in the body, e.g. liver,[70] but are most common in the kidney.
  • In the PEComa group of tumours.

Epidemiology

  • May be associated with tuberous sclerosis -- 70% have an AML.
    • When compared to sporadic cases:
      • More often bilateral.
      • Usually bigger.
  • There is a suggestion that an epithelioid variant is more worisome.[71]
    • This is not confirmed by all studies.[72]

Microscopic

Features:

  • Smooth muscle.
  • Adipose tissue - not always present[73] - key feature.
  • Abundant blood vessels.

Epithelioid angiomyolipoma

Features:

  • Carcinoma-like morphology.
  • +/-Spindle cells.
  • "High grade" nuclei.
    • Pleomorphic nuclei.

DDx:

Images:

Cytologic

Features[73]

  • Nuclei - round/ovoid.
  • Chromatin - bland.

IHC

  • Melanocytic markers +ve[75] (e.g. HMB-45 +ve).
  • Epithelial markers -ve.[75]
  • SMA +ve.
  • CD117 +ve/-ve.
  • Ki-67:[76]
    • Epithelioid variant of AML +ve.
    • Conventional AML -ve.

Mimics

Xanthogranulomatous pyelonephritis

General

  • May mimic RCC - espically radiologically.
  • Usually lower pole.[citation needed]
  • Associated with:
  • Occasionally an indication of nephrectomy.[77][78]
  • Most common organism (in the context of nephrectomy specimens) - Proteus mirabilis.[78]

Microscopic

  • Abundant macrophages.
  • +/-Giant cells.

DDx:

Image

  • Xanthogranulomatous pyelonephritis - CD68 stain. (WC/Nephron)

Stains

  • PAS-D -ve.
    • Done to look for malakoplakia.

IHC

  • CD68 +ve.
  • RCC markers (CD10, RCC) all negative.

Malakoplakia

Main article: Malakoplakia

Rare stuffs

Juxtaglomerular cell tumour

  • AKA juxtaglomerular tumour, reninoma.[79]

General

  • Rare.
  • Etiology: increased renin.

Clinical:[79]

  • Hypertension.
  • Increased aldosterone.
    • Causes hypokalemia.
  • Increased plasma renin.

Microscopic

Features:[80]

  • Polygonal cells.
  • Abundant granular, eosinophilic cytoplasm.[81]
  • Perinuclear halo.

DDx:

Image:

Stains

Cytoplasmic granules.[81]

  • PAS +ve.
  • PASD +ve.

IHC

Features:[80]

  • Actin +ve.
  • Cytokeratin -ve.
  • HMB-45 -ve.

EM

Features:

  • Vesicles - contain renin.[82]

Renomedullary interstitial cell tumour

General

  • Benign.
  • Common autopsy finding[84] - one review says 26-41% of individuals at autopsy.[85]
    • The commonality is somewhat in dispute.[86]

Gross

  • Small, white well-circumscribed nodule in medulla.
    • Typically less than 3 mm.[85]

Image:

Microscopic

Features:[84][87]

  • Small polygonal/stellate cells.
  • Abundant loose/myxoid stroma.
  • +/-Entrapped renal tubules.[88]

Images

  • Renal medullary fibroma - low mag. (WC/Nephron)

  • Renal medullary fibroma - intermed. mag. (WC/Nephron)

  • Renal medullary fibroma - very high mag. (WC/Nephron)

www:

Metanephric adenoma

  • Should not be confused mesonephric adenoma, another term for nephrogenic adenoma.
    • Memory device: metanephric adenoma is a tumour.

General

  • Benign.
  • Afflicts adults and occasionally children.
  • May be associated with polycythemia.[89]

Microscopic

Features:[90]

  • Small uniform cells with:
    • Fine chromatin.
    • No apparent nucleolus.
    • A relatively smooth nuclear membrane.
  • Variable architecture - may be sheets or ductal.

DDx:

  • Epithelioid nephroblastoma (Wilms tumour) - these typically have:
    • Irregular nuclear membrane.
    • Nucleoli.
    • Mitoses (rare in metanephric adenoma).
  • Papillary RCC.[90]

Images

  • Metanephric adenoma (WC/Nephron)

  • Metanephric adenoma - cropped (WC/Nephron)

  • Metanephric adenoma - PaRCC-like - intermed. mag. (WC/Nephron)

  • Metanephric adenoma - PaRCC-like - very high mag. (WC/Nephron)

www:

IHC

  • WT-1 +ve.
  • CD57 +ve.
  • CK7 +ve.[91] (???)
  • PAX2 +ve.[92]
  • MUC1 +ve.
    • Marks distal renal tubule.[91]

Renal epithelial and stromal tumour

Abbreviated REST.

The lumping term for both:[93]

  1. Mixed epithelial and stromal tumour.
  2. Cystic nephroma.

Mixed epithelial and stromal tumour

  • Abbreviated MEST.

General

  • Rare - approx. 1.5% of renal neoplasms.[94]
  • Benign.
  • Prevalence: males > females.

Note:

  • Turbiner et al.[93] have suggested that cystic nephroma and mixed epithelial and stromal tumour (MEST) are one tumour.
    • The lumping term renal epithelial and stromal tumour, abbreviated REST.

Microscopic

Features:

  • Cysts lined by simple epithelium with hobnailing - key feature.
  • Stroma has an ovarian look:
    • Blue (basophilic).
    • Spindle cells.

Notes:

  • Parenchymal elements (e.g. glomueruli, tubules) are not found in the septa between the cysts.

Images

  • Cystic nephroma - low mag. (WC/Nephron)

  • Cystic nephroma - intermed. mag. (WC/Nephron)

IHC

Features:[94]

  • ER +ve.
  • PR +ve.
  • CD10 +ve.

DDx

  • Tubulocystic carcinoma.

Cystic nephroma

General

Gross

  • Bubble wrap-like appearance.

Image

  • Bubble wrap. (WC)

Microscopic

Features:

  • Cysts lined by simple epithelium with hobnailing - key feature.
  • Stroma has an ovarian look:
    • Blue (basophilic).
    • Spindle cells.

Notes:

  • Parenchymal elements (e.g. glomueruli, tubules) are not found in the septa between the cysts.

Images

  • Cystic nephroma - low mag. (WC/Nephron)

  • Cystic nephroma - intermed. mag. (WC/Nephron)

IHC

Features:

  • ER +ve.
  • PR +ve.
  • CD10 +ve.

Renal mucinous tubular and spindle cell carcinoma

  • AKA renal mucinous tubular spindle cell carcinoma.
  • AKA mucinous tubular and spindle cell carcinoma of the kidney.[95]

General

  • Rare.[3]
  • Accepted by WHO in 2004 as a separate entity.[96]
  • Relatively good prognosis.

Microscopic

Features:[97]

  • Mucin - may be scant.
  • Spindle cells.

DDx:

Images:

IHC

Features:[99]

  • CD10 -ve.
  • AE1/AE3 +ve.
  • AMACR +ve.
  • CK7 +ve.

Collecting duct carcinoma

  • AKA Bellini duct carcinoma,[100] AKA carcinoma of the collecting ducts of Bellini.

General

  • Rare.
  • Poor prognosis.
  • Usu. central location.
  • Typically young adults.

Microscopic

Features:[101]

  • Tubular structures with tapered ends.
    • May be described as tubulopapillary.
  • Hobnail pattern - cell width smaller at basement membrane than free surface.[102]
  • High grade nuclear features (nuclear pleomorphism).
  • High mitotic rate.

Notes:

  • Benign urothelium must present to excluded urothelial carcinoma.
  • Desmoplastic stroma may be prominent.

DDx:

Images

www:

IHC

Features:[3]

  • HMWCK +ve.
  • LMWCK +ve.
  • CD117 +ve.
  • E-cadherin +ve.
  • CD10 -ve.
  • AMACR -ve.

UCC:

  • CK20 +ve.[3]
  • p63 +ve.
  • CK5/6 +ve.

Others:

Renal medullary carcinoma

General

  • Rare.
  • Usually young adults.
  • Associated with sickle cell trait (heterozygotes for the sickle cell allele).[103]
  • Aggressive/poor prognosis.[104]

Aside:

Gross

Features:[104]

  • Well circumscribed.
  • Renal medulla.

Microscopic

Features:[103][104]

  • Variable architecture:
  • Desmoplastic stroma - prominent.
  • Inflammation:
    • Lymphocytes.
    • Neutrophils - margination in vessels.

DDx:

Image:

IHC

Acquired cystic disease-associated renal cell carcinoma

Main article: Acquired cystic disease-associated renal cell carcinoma

Pediatric

Main article: Pediatric kidney tumours

The most common is nephroblastoma (Wilms tumour).

Others include:

See also

References

  1. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 288. ISBN 978-0781765275.
  2. ↑ Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 281-304. ISBN 978-0443066771.
  3. ↑ 3.0 3.1 3.2 3.3 3.4 Srigley, JR.; Delahunt, B. (Jun 2009). "Uncommon and recently described renal carcinomas.". Mod Pathol 22 Suppl 2: S2-S23. doi:10.1038/modpathol.2009.70. PMID 19494850. Cite error: Invalid <ref> tag; name "pmid19494850" defined multiple times with different content
  4. ↑ Tickoo, SK.; Amin, MB. (Dec 1998). "Discriminant nuclear features of renal oncocytoma and chromophobe renal cell carcinoma. Analysis of their potential utility in the differential diagnosis.". Am J Clin Pathol 110 (6): 782-7. PMID 9844591.
  5. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 291. ISBN 978-0781765275.
  6. ↑ URL:http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001544/. Accessed on: 14 July 2011.
  7. ↑ 7.0 7.1 7.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 289. ISBN 978-0781765275.
  8. ↑ Schmid HP, Szabo J (May 1997). "[Renal cell carcinoma--a current review]" (in German). Praxis (Bern 1994) 86 (20): 837?3. PMID 9312811.
  9. ↑ 9.0 9.1 9.2 Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1016. ISBN 0-7216-0187-1.
  10. ↑ Capitanio, U.; Cloutier, V.; Zini, L.; Isbarn, H.; Jeldres, C.; Shariat, SF.; Perrotte, P.; Antebi, E. et al. (Jun 2009). "A critical assessment of the prognostic value of clear cell, papillary and chromophobe histological subtypes in renal cell carcinoma: a population-based study.". BJU Int 103 (11): 1496-500. doi:10.1111/j.1464-410X.2008.08259.x. PMID 19076149.
  11. ↑ Delahunt, B.; Bethwaite, PB.; Nacey, JN. (Oct 2007). "Outcome prediction for renal cell carcinoma: evaluation of prognostic factors for tumours divided according to histological subtype.". Pathology 39 (5): 459-65. doi:10.1080/00313020701570061. PMID 17886093.
  12. ↑ Zhou M, Roma A, Magi-Galluzzi C (June 2005). "The usefulness of immunohistochemical markers in the differential diagnosis of renal neoplasms". Clin. Lab. Med. 25 (2): 247?7. doi:10.1016/j.cll.2005.01.004. PMID 15848735.
  13. ↑ 13.0 13.1 Zhou M, Roma A, Magi-Galluzzi C (June 2005). "The usefulness of immunohistochemical markers in the differential diagnosis of renal neoplasms". Clin. Lab. Med. 25 (2): 247?7. doi:10.1016/j.cll.2005.01.004. PMID 15848735.
  14. ↑ 14.0 14.1 Langner C, Ratschek M, Rehak P, Schips L, Zigeuner R (February 2004). "Expression of MUC1 (EMA) and E-cadherin in renal cell carcinoma: a systematic immunohistochemical analysis of 188 cases". Mod. Pathol. 17 (2): 180?. doi:10.1038/modpathol.3800032. PMID 14657952.
  15. ↑ 15.0 15.1 Liu L, Qian J, Singh H, Meiers I, Zhou X, Bostwick DG (August 2007). "Immunohistochemical analysis of chromophobe renal cell carcinoma, renal oncocytoma, and clear cell carcinoma: an optimal and practical panel for differential diagnosis". Arch. Pathol. Lab. Med. 131 (8): 1290?. PMID 17683191. http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=131&page=1290. Cite error: Invalid <ref> tag; name "pmid17683191" defined multiple times with different content
  16. ↑ Pan CC, Chen PC, Ho DM (November 2004). "The diagnostic utility of MOC31, BerEP4, RCC marker and CD10 in the classification of renal cell carcinoma and renal oncocytoma: an immunohistochemical analysis of 328 cases". Histopathology 45 (5): 452?. doi:10.1111/j.1365-2559.2004.01962.x. PMID 15500648.
  17. ↑ 17.0 17.1 Geramizadeh B, Ravanshad M, Rahsaz M (2008). "Useful markers for differential diagnosis of oncocytoma, chromophobe renal cell carcinoma and conventional renal cell carcinoma". Indian J Pathol Microbiol 51 (2): 167?1. PMID 18603673. http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2008;volume=51;issue=2;spage=167;epage=171;aulast=Geramizadeh.
  18. ↑ 18.0 18.1 Memeo L, Jhang J, Assaad AM, et al. (February 2007). "Immunohistochemical analysis for cytokeratin 7, KIT, and PAX2: value in the differential diagnosis of chromophobe cell carcinoma". Am. J. Clin. Pathol. 127 (2): 225–9. doi:10.1309/9KWEA4W9Y94D1AEE. PMID 17210525. http://ajcp.ascpjournals.org/cgi/pmidlookup?view=long&pmid=17210525.
  19. ↑ Mazal PR, Exner M, Haitel A, et al. (January 2005). "Expression of kidney-specific cadherin distinguishes chromophobe renal cell carcinoma from renal oncocytoma". Hum. Pathol. 36 (1): 22–8. doi:10.1016/j.humpath.2004.09.011. PMID 15712178.
  20. ↑ Krueger S, Sotlar K, Kausch I, Horny HP (2005). "Expression of KIT (CD117) in renal cell carcinoma and renal oncocytoma". Oncology 68 (2-3): 269-75. doi:10.1159/000086783. PMID 16015044.
  21. ↑ Martignoni G, Brunelli M, Gobbo S, et al (February 2007). "Role of molecular markers in diagnosis and prognosis of renal cell carcinoma". Anal. Quant. Cytol. Histol. 29 (1): 41?. PMID 17375873.
  22. ↑ Avery AK, Beckstead J, Renshaw AA, Corless CL (February 2000). "Use of antibodies to RCC and CD10 in the differential diagnosis of renal neoplasms". Am. J. Surg. Pathol. 24 (2): 203?0. PMID 10680888. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0147-5185&volume=24&issue=2&spage=203.
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  24. ↑ 24.0 24.1 de Peralta-Venturina, M.; Moch, H.; Amin, M.; Tamboli, P.; Hailemariam, S.; Mihatsch, M.; Javidan, J.; Stricker, H. et al. (Mar 2001). "Sarcomatoid differentiation in renal cell carcinoma: a study of 101 cases.". Am J Surg Pathol 25 (3): 275-84. PMID 11224597.
  25. ↑ 25.0 25.1 Lopez-Beltran, A.; Scarpelli, M.; Montironi, R.; Kirkali, Z. (May 2006). "2004 WHO classification of the renal tumors of the adults.". Eur Urol 49 (5): 798-805. doi:10.1016/j.eururo.2005.11.035. PMID 16442207.
  26. ↑ Cangiano, T.; Liao, J.; Naitoh, J.; Dorey, F.; Figlin, R.; Belldegrun, A. (Feb 1999). "Sarcomatoid renal cell carcinoma: biologic behavior, prognosis, and response to combined surgical resection and immunotherapy.". J Clin Oncol 17 (2): 523-8. PMID 10080595.
  27. ↑ 27.0 27.1 27.2 27.3 Dall'Oglio, MF.; Lieberknecht, M.; Gouveia, V.; Sant'Anna, AC.; Leite, KR.; Srougi, M.. "Sarcomatoid differentiation in renal cell carcinoma: prognostic implications.". Int Braz J Urol 31 (1): 10-6. PMID 15763002.
  28. ↑ Golshayan, AR.; George, S.; Heng, DY.; Elson, P.; Wood, LS.; Mekhail, TM.; Garcia, JA.; Aydin, H. et al. (Jan 2009). "Metastatic sarcomatoid renal cell carcinoma treated with vascular endothelial growth factor-targeted therapy.". J Clin Oncol 27 (2): 235-41. doi:10.1200/JCO.2008.18.0000. PMID 19064974.
  29. ↑ 29.0 29.1 29.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 290. ISBN 978-0781765275.
  30. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 136850
  31. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 135150
  32. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 314310
  33. ↑ Verine, J.; Pluvinage, A.; Bousquet, G.; Lehmann-Che, J.; de Bazelaire, C.; Soufir, N.; Mongiat-Artus, P. (Nov 2010). "Hereditary renal cancer syndromes: an update of a systematic review.". Eur Urol 58 (5): 701-10. doi:10.1016/j.eururo.2010.08.031. PMID 20817385.
  34. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 164860
  35. ↑ Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 282. ISBN 978-0443066771.
  36. ↑ 36.0 36.1 Delahunt, B.; Sika-Paotonu, D.; Bethwaite, PB.; McCredie, MR.; Martignoni, G.; Eble, JN.; Jordan, TW. (Jun 2007). "Fuhrman grading is not appropriate for chromophobe renal cell carcinoma.". Am J Surg Pathol 31 (6): 957-60. doi:10.1097/01.pas.0000249446.28713.53. PMID 17527087.
  37. ↑ AE. June 2010.
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  39. ↑ Agarwal, S.; Agrawal, U.; Mohanty, NK.; Saxena, S. (Mar 2011). "Multilocular cystic renal cell carcinoma: a case report of a rare entity.". Arch Pathol Lab Med 135 (3): 290-2. doi:10.1043/2010-0243-LE.1. PMID 21366448.
  40. ↑ You, D.; Shim, M.; Jeong, IG.; Song, C.; Kim, JK.; Ro, JY.; Hong, JH.; Ahn, H. et al. (Jul 2011). "Multilocular cystic renal cell carcinoma: clinicopathological features and preoperative prediction using multiphase computed tomography.". BJU Int. doi:10.1111/j.1464-410X.2011.10247.x. PMID 21722289.
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  42. ↑ Klatte, T.; Pantuck, AJ.; Said, JW.; Seligson, DB.; Rao, NP.; LaRochelle, JC.; Shuch, B.; Zisman, A. et al. (Feb 2009). "Cytogenetic and molecular tumor profiling for type 1 and type 2 papillary renal cell carcinoma.". Clin Cancer Res 15 (4): 1162-9. doi:10.1158/1078-0432.CCR-08-1229. PMID 19228721.
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  48. ↑ ALS Feb 9, 2009.
  49. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 292. ISBN 978-0781765275.
  50. ↑ 50.0 50.1 50.2 50.3 Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 293. ISBN 978-0443066771.
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  72. ↑ Aydin, H.; Magi-Galluzzi, C.; Lane, BR.; Sercia, L.; Lopez, JI.; Rini, BI.; Zhou, M. (Feb 2009). "Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association.". Am J Surg Pathol 33 (2): 289-97. doi:10.1097/PAS.0b013e31817ed7a6. PMID 18852677.
  73. ↑ 73.0 73.1 Crapanzano, JP. (Jan 2005). "Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases.". Diagn Cytopathol 32 (1): 53-7. doi:10.1002/dc.20179. PMID 15584043.
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  76. ↑ Ooi, SM.; Vivian, JB.; Cohen, RJ. (2009). "The use of the Ki-67 marker in the pathological diagnosis of the epithelioid variant of renal angiomyolipoma.". Int Urol Nephrol 41 (3): 559-65. doi:10.1007/s11255-008-9473-1. PMID 18839327.
  77. ↑ 77.0 77.1 Afgan F, Mumtaz S, Ather MH (2007). "Preoperative diagnosis of xanthogranulomatous pyelonephritis". Urol J 4 (3): 169–73. PMID 17987581.
  78. ↑ 78.0 78.1 78.2 Al-Ghazo MA, Ghalayini IF, Matalka II, Al-Kaisi NS, Khader YS (October 2006). "Xanthogranulomatous pyelonephritis: Analysis of 18 cases". Asian J Surg 29 (4): 257–61. PMID 17098659.
  79. ↑ 79.0 79.1 Wong, L.; Hsu, TH.; Perlroth, MG.; Hofmann, LV.; Haynes, CM.; Katznelson, L. (Feb 2008). "Reninoma: case report and literature review.". J Hypertens 26 (2): 368-73. doi:10.1097/HJH.0b013e3282f283f3. PMID 18192852.
  80. ↑ 80.0 80.1 80.2 Chao, CT.; Chang, FC.; Wu, VC.; Chen, JC. (Jan 2011). "Reninoma.". Kidney Int 79 (2): 260. doi:10.1038/ki.2010.445. PMID 21191395.
  81. ↑ 81.0 81.1 Hanna, W.; Tepperman, B.; Logan, AG.; Robinette, MA.; Colapinto, R.; Phillips, MJ. (Apr 1979). "Juxtaglomerular cell tumour (reninoma) with paroxysmal hypertension.". Can Med Assoc J 120 (8): 957-9. PMID PMC = 1819229 436071 PMC = 1819229.
  82. ↑ URL: http://path.upmc.edu/cases/case29/micro.html. Accessed on: 18 December 2011.
  83. ↑ Bircan, S.; Orhan, D.; Tulunay, O.; Safak, M. (2000). "Renomedullary interstitial cell tumor.". Urol Int 65 (3): 163-6. PMID 11054036.
  84. ↑ 84.0 84.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 295. ISBN 978-0781765275.
  85. ↑ 85.0 85.1 Tsurukawa, H.; Iuchi, H.; Osanai, H.; Yamaguchi, S.; Hashimoto, H.; Kaneko, S.; Yachiku, S. (Jan 2000). "[Renomedullary interstitial cell tumor: a case report].". Nihon Hinyokika Gakkai Zasshi 91 (1): 37-40. PMID 10689882.
  86. ↑ Kozłowska, J.; Okoń, K. (2008). "Renal tumors in postmortem material.". Pol J Pathol 59 (1): 21-5. PMID 18655367.
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