Kidney Cancer: Pathology: Difference between revisions
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== Classification of renal masses == | == Classification of renal masses == | ||
=== | === 2016 WHO Classification of renal neoplasms[https://pubmed.ncbi.nlm.nih.gov/26935559/] === | ||
(abbreviated) | |||
{| class="wikitable" | {| class="wikitable" | ||
| | | | ||
* '''Renal cell tumours (16)''' | * '''<span style="color:#ff0000">Renal cell tumours (16)</span>''' | ||
** Clear cell renal cell carcinoma | ** Clear cell renal cell carcinoma | ||
** Multilocular cystic renal neoplasm of low malignant potential | ** Multilocular cystic renal neoplasm of low malignant potential | ||
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** Metanephric adenofibroma | ** Metanephric adenofibroma | ||
** Metanephric stromal tumor | ** Metanephric stromal tumor | ||
* '''Nephroblastic and cystic tumors occurring mainly in children''' | * '''Nephroblastic and cystic tumors occurring mainly in children''' | ||
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** Pediatric cystic nephroma | ** Pediatric cystic nephroma | ||
| | | | ||
* Mesenchymal tumors occurring mainly in children | * '''Mesenchymal tumors occurring mainly in children''' | ||
** Clear cell sarcoma | ** Clear cell sarcoma | ||
** Rhabdoid tumor | ** Rhabdoid tumor | ||
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** Ossifying renal tumor of infancy | ** Ossifying renal tumor of infancy | ||
* Mesenchymal tumors occurring mainly in adults | * '''Mesenchymal tumors occurring mainly in adults''' | ||
** Leiomyosarcoma (including renal vein leiomyosarcoma) | ** Leiomyosarcoma (including renal vein leiomyosarcoma) | ||
** Angiosarcoma | ** Angiosarcoma | ||
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** Mixed epithelial and stromal tumor | ** Mixed epithelial and stromal tumor | ||
* Neuroendocrine tumors | * '''Neuroendocrine tumors''' | ||
** Well differentiated neuroendocrine tumor | ** Well differentiated neuroendocrine tumor | ||
** Large cell neuroendocrine carcinoma | ** Large cell neuroendocrine carcinoma | ||
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** Paraganglioma | ** Paraganglioma | ||
* Renal hematopoietic neoplasms | * '''Miscellaneous tumors''' | ||
**Renal hematopoietic neoplasms | |||
* Germ cell tumors | **Germ cell tumors | ||
* Metastatic tumors | * '''Metastatic tumors''' | ||
* Tumor-like lesions | * '''Tumor-like lesions''' | ||
** Xanthogranulomatous pyelonephritis | ** Xanthogranulomatous pyelonephritis | ||
** IgG4 related disease | ** IgG4 related disease | ||
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There are additional described entities not currently in WHO | There are additional described entities not currently in WHO | ||
=== | === Classification by malignant vs. benign vs. inflammatory === | ||
{| class="wikitable" | {| class="wikitable" | ||
| | | | ||
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== Renal cell carcinoma (RCC) == | == Renal cell carcinoma (RCC) == | ||
* '''Most common (>90%) non-metastatic malignant histology of kidney tumours''' | * '''<span style="color:#ff0000">Most common (>90%) non-metastatic malignant histology of kidney tumours</span>''' | ||
** Most common benign tumours of the kidney include oncocytoma and angiomyolipoma (AML). | ** Most common benign tumours of the kidney include oncocytoma and angiomyolipoma (AML). | ||
* '''Most common renal tumour in pregnancy''' | * '''<span style="color:#ff0000">Most common renal tumour in pregnancy[https://pubmed.ncbi.nlm.nih.gov/3756780/]</span>''' | ||
* '''All RCCs are adenocarcinomas''' | * '''<span style="color:#ff0000">All RCCs are adenocarcinomas</span>''' | ||
** '''Most are derived from renal tubular epithelial cells of the proximal convoluted tubule''' | ** '''<span style="color:#ff0000">Most are derived from</span> renal tubular epithelial cells <span style="color:#ff0000">of the proximal convoluted tubule</span>''' | ||
*** Exceptions include chromophobe, collecting duct, and medullary RCC (see below) | *** Exceptions include chromophobe, collecting duct, and medullary RCC (see below) | ||
=== | === <span style="color:#ff0000">Subtypes</span> === | ||
{| class="wikitable" | {| class="wikitable" | ||
|'''Histology''' | |'''<span style="color:#ff0000">Histology</span>''' | ||
|'''Characteristics''' | |'''<span style="color:#ff0000">Characteristics</span>''' | ||
|'''Familial form and genetic factors''' | |'''<span style="color:#ff0000">Familial form and genetic factors</span>''' | ||
|- | |- | ||
|'''Clear cell RCC (ccRCC)''' | |'''<span style="color:#ff0000">Clear cell RCC (ccRCC)</span>''' | ||
'''(70-80%, most common)''' | '''<span style="color:#ff0000">(70-80%, most common)</span>''' | ||
|'''Originates from proximal tubule''' | |'''<span style="color:#ff0000">Originates from proximal tubule</span>''' | ||
'''Prognosis generally worse compared to papillary or chromophobe''' | '''<span style="color:#ff0000">Prognosis generally worse compared to papillary or chromophobe</span>''' | ||
'''Responds to systemic therapy''' | '''Responds to systemic therapy''' | ||
|'''von Hippel-Lindau disease''' | |'''<span style="color:#ff0000">von Hippel-Lindau disease</span>''' | ||
'''Chromosome 3p deletions''' (VHL inactivation by mutation or promoter hypermethylation occurs in 70-90% of clear cell renal tumors) | '''Chromosome 3p deletions''' (VHL inactivation by mutation or promoter hypermethylation occurs in 70-90% of clear cell renal tumors) | ||
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|'''Identical to ccRCC''' | |'''Identical to ccRCC''' | ||
|- | |- | ||
|'''Papillary RCC''' | |'''<span style="color:#ff0000">Papillary RCC</span>''' | ||
'''(10-15%, 2nd most common)''' | '''<span style="color:#ff0000">(10-15%, 2nd most common)</span>''' | ||
|'''Originates from proximal tubule''' | |'''<span style="color:#ff0000">Originates from proximal tubule</span>''' | ||
'''Commonly multifocal''' | '''<span style="color:#ff0000">Commonly multifocal</span>''' | ||
'''Common in ESRD and acquired renal cystic disease''' | '''<span style="color:#ff0000">Common in ESRD and acquired renal cystic disease</span>''' | ||
'''Type 1: good prognosis''' | '''<span style="color:#ff0000">Type 1: good prognosis</span>''' | ||
'''Type 2: worse prognosis''' | '''<span style="color:#ff0000">Type 2: worse prognosis</span>''' | ||
'''Grade may be of greater prognostic significance than type 1 vs. 2''' | '''Grade may be of greater prognostic significance than type 1 vs. 2''' | ||
'''Current systemic therapies are ineffective against papillary RCC''' | '''<span style="color:#ff0000">Current systemic therapies are ineffective against papillary RCC</span>''' | ||
Papillary adenomas are small (≤5mm) tumours that resemble papillary RCC under the microscope, are often well encapsulated and low grade, commonly found at autopsy, possess many of the same genetic alterations found in larger papillary RCCs, but are benign neoplasms | Papillary adenomas are small (≤5mm) tumours that resemble papillary RCC under the microscope, are often well encapsulated and low grade, commonly found at autopsy, possess many of the same genetic alterations found in larger papillary RCCs, but are benign neoplasms | ||
|'''Type 1: Hereditary papillary RCC (HPRCC) syndrome''' | |'''<span style="color:#ff0000">Type 1: Hereditary papillary RCC (HPRCC) syndrome</span>''' | ||
'''Type 2: Hereditary leiomyomatosis and RCC syndrome (HLRCC)''' | '''<span style="color:#ff0000">Type 2: Hereditary leiomyomatosis and RCC syndrome (HLRCC)</span>''' | ||
'''Trisomy of chromosomes 7 and 17 and loss of the Y chromosome.''' | '''Trisomy of chromosomes 7 and 17 and loss of the Y chromosome.''' | ||
|- | |- | ||
|'''Chromophobe RCC''' | |'''<span style="color:#ff0000">Chromophobe RCC</span>''' | ||
'''(3-5%)''' | '''<span style="color:#ff0000">(3-5%)</span>''' | ||
|'''Originates from''' intercalated cells of '''distal tubule/collecting duct''' | |'''<span style="color:#ff0000">Originates from</span>''' intercalated cells of '''<span style="color:#ff0000">distal tubule/collecting duct</span>''' | ||
'''Stains positive for Hale colloidal iron''' | '''Stains positive for Hale colloidal iron''' | ||
''' | '''<span style="color:#ff0000">Generally good prognosis</span>''', compared to clear cell and papillary | ||
* Rates of disease-specific (recurrence, metastasis, or death due disease) events following nephrectomy: | * Rates of disease-specific (recurrence, metastasis, or death due disease) events following nephrectomy:[https://pubmed.ncbi.nlm.nih.gov/21602658/] | ||
** 5 years: 3.7% | ** 5 years: 3.7% | ||
** 10 years: 6.4% | ** 10 years: 6.4% | ||
* Features associated with disease-specific events (4): | * Features associated with disease-specific events (4):[https://pubmed.ncbi.nlm.nih.gov/21602658/] | ||
*# Tumour size | *# Tumour size | ||
*# Small-vessel invasion | *# Small-vessel invasion | ||
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*# Microscopic necrosis | *# Microscopic necrosis | ||
** pT stage or nodal metastasis tended to show some association, without reaching statistical significance | ** pT stage or nodal metastasis tended to show some association, without reaching statistical significance | ||
|'''Commonly seen Birt-Hogg-Dubé syndrome; most cases are sporadic''' | |'''<span style="color:#ff0000">Commonly seen Birt-Hogg-Dubé syndrome;</span> most cases are sporadic''' | ||
|- | |- | ||
|'''Collecting duct carcinoma (<1%)''' | |'''<span style="color:#ff0000">Collecting duct carcinoma (<1%)</span>''' | ||
|'''Originates from collecting duct''' | |'''<span style="color:#ff0000">Originates from collecting duct</span>''' | ||
'''Stains positive with Ulex europaeus lectin''' | '''Stains positive with Ulex europaeus lectin''' | ||
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Multiple chromosomal losses | Multiple chromosomal losses | ||
|- | |- | ||
|'''Renal medullary carcinoma (rare)''' | |'''<span style="color:#ff0000">Renal medullary carcinoma</span> (rare)''' | ||
|'''Originates from collecting duct''' | |'''<span style="color:#ff0000">Originates from collecting duct</span>''' | ||
'''Dismal prognosis'''; '''many cases are both locally advanced and metastatic at the time of diagnosis''' | '''Dismal prognosis'''; '''many cases are both locally advanced and metastatic at the time of diagnosis''' | ||
|'''Associated with sickle cell trait (NOT disease);''' typically diagnosed in young African-Americans | |'''<span style="color:#ff0000">Associated with sickle cell trait (NOT disease);</span>''' typically diagnosed in young African-Americans | ||
|- | |- | ||
|'''Unclassified RCC''' | |'''Unclassified RCC''' | ||
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|} | |} | ||
=== | === <span style="color:#ff0000">Other histologic features</span> === | ||
* '''Sarcomatoid differentiation''' | * '''<span style="color:#ff0000">Sarcomatoid differentiation</span>''' | ||
** Found in 1-5% of RCCs | ** Found in 1-5% of RCCs | ||
** '''Not a distinct histologic subtype of RCC''', '''most commonly in association with ccRCC and chromophobe RCC''' | ** '''Not a distinct histologic subtype of RCC''', '''<span style="color:#ff0000">most commonly in association with ccRCC and chromophobe RCC</span>''' | ||
** '''Associated with worse prognosis; multimodal approaches should be considered''' | ** '''<span style="color:#ff0000">Associated with worse prognosis;</span> multimodal approaches should be considered''' | ||
* '''Cystic degeneration''' | * '''Cystic degeneration''' | ||
** Found in 10-25% of RCCs | ** Found in 10-25% of RCCs | ||
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* '''Laterality and focality''' | * '''Laterality and focality''' | ||
** '''Most sporadic RCCs are unilateral and unifocal''' | ** '''<span style="color:#ff0000">Most sporadic RCCs are unilateral and unifocal</span>''' | ||
** '''Bilateral involvement''' | ** '''<span style="color:#ff0000">Bilateral involvement</span>''' | ||
*** Occurs in 2-4% of sporadic RCCs | *** Occurs in 2-4% of sporadic RCCs | ||
**** '''More common in patients with familial forms of RCC ('''e.g'''.''' von Hippel-Lindau disease). | **** '''More common in patients with familial forms of RCC ('''e.g'''.''' von Hippel-Lindau disease). | ||
*** Can be synchronous or asynchronous | *** Can be synchronous or asynchronous | ||
**** '''If synchronous, likely an independent growth''' | **** '''<span style="color:#ff0000">If synchronous, likely an independent growth</span>''' | ||
**** '''If asynchronous, likely a metastasis''' | **** '''<span style="color:#ff0000">If asynchronous, likely a metastasis</span>''' | ||
** '''Multifocality''' | ** '''<span style="color:#ff0000">Multifocality''' | ||
*** '''Occurs in 10-20% of cases''' | *** '''<span style="color:#ff0000">Occurs in 10-20% of cases</span>''' | ||
*** '''More common with papillary histology and familial RCC''' | *** '''<span style="color:#ff0000">More common with papillary histology and familial RCC</span>''' | ||
*** '''Microsatellite analysis suggests a clonal origin for most multifocal RCC | *** '''<span style="color:#ff0000">Microsatellite analysis suggests a clonal origin for most multifocal RCC</span>''' | ||
== Grade == | == Grade == | ||
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** Sarcomatoid and rhabdoid tumors, tumors with giant cells, and tumors with extreme nuclear pleomorphism are included within grade 4 tumors. | ** Sarcomatoid and rhabdoid tumors, tumors with giant cells, and tumors with extreme nuclear pleomorphism are included within grade 4 tumors. | ||
** Chromophobe RCC is no longer graded in the ISUP system. | ** Chromophobe RCC is no longer graded in the ISUP system. | ||
* In general, higher grade is associated with larger tumor size and more aggressive tumors. | * In general, higher grade is associated with larger tumor size and more aggressive tumors.</span> | ||
== Questions == | == Questions == | ||
# What the prognosis of ccRCC relative to chromophobe and papillary RCC? Which papillary RCC subtype is associated with better prognosis relative to the other? | # What the prognosis of ccRCC relative to chromophobe and papillary RCC? Which papillary RCC subtype is associated with better prognosis relative to the other? | ||
# Patients with ESRD or acquired renal cystic disease are more likely to develop | # Patients with ESRD or acquired renal cystic disease are more likely to develop which type of RCC? | ||
# Which RCC histology stains for Hale colloidal iron? | # Which RCC histology stains for Hale colloidal iron? | ||
# Which RCC histologies arise from the proximal tubule vs. collecting duct? | # Which RCC histologies arise from the proximal tubule vs. collecting duct? | ||
== Answers == | == Answers == | ||
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### Collecting duct | ### Collecting duct | ||
### Medullary | ### Medullary | ||
== Next Chapter: TNM Staging == | == Next Chapter: [[Kidney Cancer: TNM Staging|TNM Staging]] == | ||
== References == | == References == |
Latest revision as of 11:03, 17 March 2024
Classification of renal masses[edit | edit source]
2016 WHO Classification of renal neoplasms[1][edit | edit source]
(abbreviated)
|
|
There are additional described entities not currently in WHO
Classification by malignant vs. benign vs. inflammatory[edit | edit source]
|
|
Renal cell carcinoma (RCC)[edit | edit source]
- Most common (>90%) non-metastatic malignant histology of kidney tumours
- Most common benign tumours of the kidney include oncocytoma and angiomyolipoma (AML).
- Most common renal tumour in pregnancy[2]
- All RCCs are adenocarcinomas
- Most are derived from renal tubular epithelial cells of the proximal convoluted tubule
- Exceptions include chromophobe, collecting duct, and medullary RCC (see below)
- Most are derived from renal tubular epithelial cells of the proximal convoluted tubule
Subtypes[edit | edit source]
Histology | Characteristics | Familial form and genetic factors |
Clear cell RCC (ccRCC)
(70-80%, most common) |
Originates from proximal tubule
Prognosis generally worse compared to papillary or chromophobe Responds to systemic therapy |
von Hippel-Lindau disease
Chromosome 3p deletions (VHL inactivation by mutation or promoter hypermethylation occurs in 70-90% of clear cell renal tumors) |
Multilocular cystic
ccRCC (uncommon) |
Almost uniformly benign clinical behavior | Identical to ccRCC |
Papillary RCC
(10-15%, 2nd most common) |
Originates from proximal tubule
Commonly multifocal Common in ESRD and acquired renal cystic disease Type 1: good prognosis Type 2: worse prognosis Grade may be of greater prognostic significance than type 1 vs. 2 Current systemic therapies are ineffective against papillary RCC Papillary adenomas are small (≤5mm) tumours that resemble papillary RCC under the microscope, are often well encapsulated and low grade, commonly found at autopsy, possess many of the same genetic alterations found in larger papillary RCCs, but are benign neoplasms |
Type 1: Hereditary papillary RCC (HPRCC) syndrome
Type 2: Hereditary leiomyomatosis and RCC syndrome (HLRCC) Trisomy of chromosomes 7 and 17 and loss of the Y chromosome. |
Chromophobe RCC
(3-5%) |
Originates from intercalated cells of distal tubule/collecting duct
Stains positive for Hale colloidal iron Generally good prognosis, compared to clear cell and papillary
|
Commonly seen Birt-Hogg-Dubé syndrome; most cases are sporadic |
Collecting duct carcinoma (<1%) | Originates from collecting duct
Stains positive with Ulex europaeus lectin Poor prognosis; most reported cases have been high grade, advanced stage, and unresponsive to conventional therapies May share features in common with urothelial carcinoma; advanced collecting duct carcinoma may respond to cisplatin or gemcitabine-based chemotherapy |
Unknown
Multiple chromosomal losses |
Renal medullary carcinoma (rare) | Originates from collecting duct
Dismal prognosis; many cases are both locally advanced and metastatic at the time of diagnosis |
Associated with sickle cell trait (NOT disease); typically diagnosed in young African-Americans |
Unclassified RCC
(1%-3%) |
Origin not defined
Poor prognosis, most are poorly differentiated and are associated with a highly aggressive biologic behavior |
Unknown |
RCC associated with Xp11.2
translocations/TFE3 gene fusions (rare) |
Occurs in children and young adults; 40% of pediatric RCC
Prognosis similar to ccRCC |
Various mutations involving chromosome Xp11.2
resulting in TFE3 gene fusion |
Post-neuroblastoma
RCC (rare) |
Occurs exclusively in children with prior neuroblastoma | Unknown |
Mucinous tubular and spindle cell (rare) | Favorable prognosis | Unknown |
Other histologic features[edit | edit source]
- Sarcomatoid differentiation
- Found in 1-5% of RCCs
- Not a distinct histologic subtype of RCC, most commonly in association with ccRCC and chromophobe RCC
- Associated with worse prognosis; multimodal approaches should be considered
- Cystic degeneration
- Found in 10-25% of RCCs
- Associated with better prognosis compared with purely solid RCC
- Laterality and focality
- Most sporadic RCCs are unilateral and unifocal
- Bilateral involvement
- Occurs in 2-4% of sporadic RCCs
- More common in patients with familial forms of RCC (e.g. von Hippel-Lindau disease).
- Can be synchronous or asynchronous
- If synchronous, likely an independent growth
- If asynchronous, likely a metastasis
- Occurs in 2-4% of sporadic RCCs
- Multifocality
- Occurs in 10-20% of cases
- More common with papillary histology and familial RCC
- Microsatellite analysis suggests a clonal origin for most multifocal RCC
Grade[edit | edit source]
- Based primarily on nuclear size, shape, and presence of predominant nucleoli
- Fuhrman Grading system
- Described in 1982
- International Society of Urological Pathology (ISUP) Grading system
- Proposed in 2012, updated in 2016
- Incorporates aspects of the Fuhrman Grading system but includes more objective criteria for nuclear characteristics.
- Sarcomatoid and rhabdoid tumors, tumors with giant cells, and tumors with extreme nuclear pleomorphism are included within grade 4 tumors.
- Chromophobe RCC is no longer graded in the ISUP system.
- In general, higher grade is associated with larger tumor size and more aggressive tumors.
Questions[edit | edit source]
- What the prognosis of ccRCC relative to chromophobe and papillary RCC? Which papillary RCC subtype is associated with better prognosis relative to the other?
- Patients with ESRD or acquired renal cystic disease are more likely to develop which type of RCC?
- Which RCC histology stains for Hale colloidal iron?
- Which RCC histologies arise from the proximal tubule vs. collecting duct?
Answers[edit | edit source]
- What the prognosis of ccRCC relative to chromophobe and papillary RCC? Which papillary RCC subtype is associated with better prognosis relative to the other?
- Prognosis of ccRCC is worse than chromophobe and papillary RCC
- Type II papillary RCC has worse prognosis than type I
- Patients with ESRD or acquired renal cystic disease are more likely to develop with type of RCC?
- Papillary
- Which RCC histology stains for Hale colloidal iron?
- Chromophobe
- Which RCC histologies arise from the proximal tubule vs. collecting duct?
- Proximal tubule:
- Clear cell
- Papillary
- Collecting duct
- Chromophobe
- Collecting duct
- Medullary
- Proximal tubule:
Next Chapter: TNM Staging[edit | edit source]
References[edit | edit source]
- Wein AJ, Kavoussi LR, Partin AW, Peters CA (eds): CAMPBELL-WALSH UROLOGY, ed 11. Philadelphia, Elsevier, 2015, chap 57
- Campbell, Steven C., et al. "Renal Mass and Localized Renal Cancer: Evaluation, Management, and Follow-Up: AUA Guideline Part I." The Journal of urology (2021): 10-1097.