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==Overview==
==Overview==
Renal cell carcinoma may be classified according to [[histologic]] appearance of the tumor into 6 subtypes: conventional (clear cell), [[papillary]], [[chromophobe]], [[oncocytoma]], [[collecting duct]], and unclassified.
==Classification==
==Classification==
===Sporadic vs. Hereditary Renal Cell Carcinoma===
The following table classifies renal cell carcinoma according to histologic appearance and differentiates sporadic from hereditary forms of renal cell carcinoma. Clear type (also called Conventional type) renal cell carcinoma is considered the most common type of renal carcinoma.
The following tables classify renal cell carcinoma according to histologic appearance and differentiates sporadic from hereditary forms of renal cell carcinoma. Clear type (also called Conventional type) renal cell carcinoma is considered the most common type of renal carcinoma.
The most common classification of renal cell carcinoma is based on the [[histopathological]] appearance of the [[tumor]]. There are currently six subtypes of renal cell carcinoma, most common of which are the conventional (also called clear cell) renal carcinoma, accounting for approximately 75% of all cases.<ref name="pmid16339096">{{cite journal| author=Cohen HT, McGovern FJ| title=Renal-cell carcinoma. |journal=N Engl J Med | year= 2005 | volume= 353 | issue= 23 | pages= 2477-90 | pmid=16339096 |doi=10.1056/NEJMra043172 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16339096  }} </ref> Renal cell carcinoma may also be clinically classified based on the staging of the tumor, as shown below.
====Sporadic Forms of Renal Cell Carcinoma====
{| border="1" style="border-collapse:collapse; text-align:left;" cellpadding="5" align="center"
|+ '''''Sporadic Forms of Renal Cell Carcinoma<ref name="pmid16339096">{{cite journal| author=Cohen HT, McGovern FJ| title=Renal-cell carcinoma. | journal=N Engl J Med | year= 2005 | volume= 353 | issue= 23 | pages= 2477-90 | pmid=16339096 | doi=10.1056/NEJMra043172 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16339096  }} </ref>'''''
| bgcolor="#d9ff54"|'''Histologic Appearance''' || bgcolor="#d9ff54"|'''Incidence'''||bgcolor="#d9ff54"|'''Gene'''||bgcolor="#d9ff54"|'''Frequency'''
|-
| bgcolor="#ececec"|'''Conventional (Clear Cell)''' || 75 || VHL|| 60 %  
|-
| bgcolor="#ececec"|'''Papillary''' || 12|| MET <br> TFE3|| 13 %<br> < 1 %
|-
| bgcolor="#ececec"|'''Chromophobe''' || 4  ||  ||
|-
| bgcolor="#ececec"|'''Oncocytoma''' || 4 ||  ||
|-
|bgcolor="#ececec"|'''Collecting Duct''' || < 1 ||  ||
|-
|bgcolor="#ececec"|'''Unclassified''' || 3 - 5 ||  ||
|}
<sup><center>Adapted from Cohen HT, McGovern FJ. Renal-cell carcinoma.''N Engl J Med''. 2005; 353:2477-90</center></sup>
 
====Hereditary Forms of Renal Cell Carcinoma====
{| border="1" style="border-collapse:collapse; text-align:left;" cellpadding="5" align="center"
|+ '''''Hereditary Forms of Renal Cell Carcinoma<ref name="pmid16339096">{{cite journal|author=Cohen HT, McGovern FJ| title=Renal-cell carcinoma. | journal=N Engl J Med |year= 2005 | volume= 353 | issue= 23 | pages= 2477-90 | pmid=16339096 |doi=10.1056/NEJMra043172 | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16339096  }}</ref>'''''
 


| bgcolor="#d9ff54"|'''Syndrome''' || bgcolor="#d9ff54"|'''Associated Gene'''||bgcolor="#d9ff54"|'''Common Histological Appearance'''
===Histologic Appearance===
The following table summarizes the incidence of various histological types of renal tumors:
{| style="border-collapse:collapse; text-align:left;" align="center" cellpadding="5" border="1"
|+'''''Heidelberg Classification Renal Cell Carcinoma According to Histological Appearance (1997)<ref name="pmid16339096">{{cite journal| author=Cohen HT, McGovern FJ| title=Renal-cell carcinoma. | journal=N Engl J Med | year= 2005 | volume= 353 | issue= 23 | pages= 2477-90 | pmid=16339096 | doi=10.1056/NEJMra043172 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16339096  }} </ref><ref name="pmid9390023">{{cite journal| author=Kovacs G, Akhtar M, Beckwith BJ, Bugert P, Cooper CS, Delahunt B et al.| title=The Heidelberg classification of renal cell tumours. | journal=J Pathol | year= 1997 | volume= 183 | issue= 2 | pages= 131-3 | pmid=9390023 | doi=10.1002/(SICI)1096-9896(199710)183:2<131::AID-PATH931>3.0.CO;2-G | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9390023  }} </ref>'''''
| style="background:#4479BA; color: #FFFFFF;" align="center" |'''Histologic Appearance'''|| style="background:#4479BA; color: #FFFFFF;" align="center" |'''Incidence (%)'''
|-
|-
| bgcolor="#ececec"|'''VHL Disease''' || VHL || Conventional (Clear Cell)
| bgcolor="#D5D5D5" |'''Conventional (Clear Cell)'''|| 75
|-
|-
| bgcolor="#ececec"|'''FCRC''' || Chromosome 3p translocation || Conventional (Clear Cell)
| bgcolor="#D5D5D5" |'''Papillary'''|| 12
|-
|-
| bgcolor="#ececec"|'''Hereditary Paraganglioma''' || SDHB || Conventional (Clear Cell)
| bgcolor="#D5D5D5" |'''Chromophobe'''|| 4
|-
|-
| bgcolor="#ececec"|'''HPRC''' || MET || Papillary
| bgcolor="#D5D5D5" |'''Oncocytoma'''|| 4
|-
|-
| bgcolor="#ececec"|'''HLRCC''' || FH || Papillary
| bgcolor="#D5D5D5" |'''Collecting Duct'''|| < 1
|-
|-
|bgcolor="#ececec"|'''Birt-Hogg-Dube Syndrome''' || BHD || Chromophobe / Oncocytoma
| bgcolor="#D5D5D5" |'''Unclassified'''|| 3 - 5
|}
|}
<sup><center>Adapted from Cohen HT, McGovern FJ. Renal-cell carcinoma.''N Engl J Med''. 2005; 353:2477-90</center></sup>
<sup><center>Adapted from Cohen HT, McGovern FJ. Renal-cell carcinoma.''N Engl J Med''. 2005; 353:2477-90</center></sup>


===Staging of Renal Clear Cell Carcinoma===
More than 70% of renal cell carcinomas are clear cell carcinomas. Papillary carcinomas are the second most common renal cell carcinomas. The least common types of renal cell carcinomas are [[Collecting duct system|collecting-duct]] renal cell carcinomas, accounting for less than 1% of all cases.<ref name="pmid16339096">{{cite journal| author=Cohen HT, McGovern FJ| title=Renal-cell carcinoma. | journal=N Engl J Med | year= 2005 | volume= 353 | issue= 23 | pages= 2477-90 | pmid=16339096 | doi=10.1056/NEJMra043172 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16339096 }} </ref>
Clear cell renal carcinomas can be classified according to staging or anatomic spread of the disease. Two commonly implemented systems are the Modified Robson's System (1969) and the TNM (Tumor - Nodes - Metastasis) System:
 
====Robson's System====
The Modified Robson's staging system has been proposed in 1969. It emphasizes the anatomic distribution of the tumor and proximity to important vasculature and lymph nodes with prognostic implications, reporting a 52% 5-year survival and a 66% 5-year survival in patients with localized tumor.<ref name="pmid5765875">{{cite journal| author=Robson CJ, Churchill BM, Anderson W| title=The results of radical nephrectomy for renal cell carcinoma. | journal=J Urol | year= 1969 | volume= 101 | issue= 3 | pages= 297-301 | pmid=5765875 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5765875  }} </ref>
 
{| border="1" style="border-collapse:collapse; text-align:left;" cellpadding="5" align="center"
|+ '''''Robson's Classification System of Renal Clear Cell Carcinoma Staging<ref name="pmid5765875">{{cite journal| author=Robson CJ, Churchill BM, Anderson W| title=The results of radical nephrectomy for renal cell carcinoma. | journal=J Urol | year= 1969 | volume= 101 | issue= 3 | pages= 297-301 | pmid=5765875 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5765875  }} </ref>'''''
 
| bgcolor="#d9ff54"|'''Stage''' || bgcolor="#d9ff54"|'''Definition
|-
| bgcolor="#ececec"|'''Stage I''' || Tumor confined within parenchyma
|-
| bgcolor="#ececec"|'''Stage II''' || Tumor involves perinephric fat, but still confined within Gerota's fascia
|-
| bgcolor="#ececec"|'''Stage III''' || A) Tumor involves main renal vein or vena cava  <br> B) Tumor involves regional lymph nodes  <br> C) Tumor involves both local vessels and lymph nodes
|-
| bgcolor="#ececec"|'''Stage IV''' || A) Tumor involves adjacent organs other than adrenal gland <br> B) Distant metastasis
|}
<sup><center>Adapted from Robson CJ, Churchill BM, Anderson W. The results of radical nephrectomy for renal cell carcinoma. ''J Urol''. 1969; 101(3):297-301.</center></sup>
 
====TNM System====
The TNM classification system for staging of renal clear cell carcinoma is commonly used. The following TNM classification is based on the American Joint Committee Cancer (AJCC), the American College of Radiology (ACR), and the Union for International Cancer Control (UICC).
 
{| border="1" style="border-collapse:collapse; text-align:left;" cellpadding="5" align="center"
|+ ''''' TNM Classification System of Renal Clear Cell Carcinoma Staging'''''
 
| bgcolor="#d9ff54"|'''T.N.M''' || bgcolor="#d9ff54"|'''Stage'''
|-
| bgcolor="#ececec"|'''Primary Tumor (T)''' || '''TX''': Primary tumor cannot be assessed <br> '''T0''': No evidence of primary tumor <br>'''T1''':  Tumor 7 cm or less in greatest dimension, limited to the kidney <br> '''T1a''': Tumor 4 cm or less in greatest dimension, limited to the kidney <br> '''T1b''': Tumor more than 4 cm but not more than 7 cm in greatest dimension, limited to the kidney <br> '''T2''': Tumor more than 7 cm in greatest dimension, limited to the kidney <br> '''T2a''': Tumor more than 7 cm but less than or equal to 10 cm in greatest dimension, limited to the kidney <br> '''T2b''': Tumor more than 10 cm, limited to the kidney <br> '''T3''': Tumor extends into major veins or perinephric tissues, but not into the ipsilateral adrenal gland and not beyond Gerota's fascia <br> '''T3a''': Tumor grossly extends into the renal vein or its segmental (muscle containing) branches, or tumor invades perirenal and/or renal sinus fat but not beyond Gerota's fascia <br> '''T3b''': Tumor grossly extends into the vena cava below the diaphragm <br> '''T3c''': Tumor grossly extends into the vena cava above the diaphragm or invades the wall of the vena cava'''T4''': Tumor invades beyond Gerota's fascia (including contiguous extension into the ipsilateral adrenal gland)
|-
| bgcolor="#ececec"|'''Regional Lymph Nodes (N)''' || '''NX''': Regional lymph nodes (LN) cannot be assessed <br>'''N0''': No regional LN metastases <br> '''N1''': Metastasis in a single LN 2 cm or less  <br> '''N2''': Metastasis in a single LN  greater than 2 cm, but less than 5 cm, or multiple LN none greater than 5 cm <br> '''N3''': Metastasis in LN greater than 5 cm
|-
| bgcolor="#ececec"|'''Distant Metastasis (M)''' || '''M0''': No distant metastasis <br> '''M1''': Distant metastasis
|}
 
To note, proposals in December 2013 to amend the TNM staging of renal clear cell carcinoma have emerged following a 41.7 month follow-up of 122 patients with pT3a renal clear cell carcinoma.<ref name="pmid23816525">{{cite journal| author=Baccos A, Brunocilla E, Schiavina R, Borghesi M, Rocca GC, Chessa F et al.| title=Differing risk of cancer death among patients with pathologic t3a renal cell carcinoma: identification of risk categories according to fat infiltration and renal vein thrombosis. | journal=Clin Genitourin Cancer | year= 2013 | volume= 11 | issue= 4 | pages= 451-7 | pmid=23816525 | doi=10.1016/j.clgc.2013.05.006 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23816525 }} </ref> Baccos and colleagues concluded that fat and/or renal vein invasion are important prognostic factors that play a major role in patient survival and should be considered in the TNM staging. Patients with both fat invasion and renal vein thrombosis have worse survival rates than those with only one element.


==References==
==References==
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Latest revision as of 16:38, 9 October 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Renal cell carcinoma may be classified according to histologic appearance of the tumor into 6 subtypes: conventional (clear cell), papillary, chromophobe, oncocytoma, collecting duct, and unclassified.

Classification

The following table classifies renal cell carcinoma according to histologic appearance and differentiates sporadic from hereditary forms of renal cell carcinoma. Clear type (also called Conventional type) renal cell carcinoma is considered the most common type of renal carcinoma. The most common classification of renal cell carcinoma is based on the histopathological appearance of the tumor. There are currently six subtypes of renal cell carcinoma, most common of which are the conventional (also called clear cell) renal carcinoma, accounting for approximately 75% of all cases.[1] Renal cell carcinoma may also be clinically classified based on the staging of the tumor, as shown below.

Histologic Appearance

The following table summarizes the incidence of various histological types of renal tumors:

Heidelberg Classification Renal Cell Carcinoma According to Histological Appearance (1997)[1][2]
Histologic Appearance Incidence (%)
Conventional (Clear Cell) 75
Papillary 12
Chromophobe 4
Oncocytoma 4
Collecting Duct < 1
Unclassified 3 - 5
Adapted from Cohen HT, McGovern FJ. Renal-cell carcinoma.N Engl J Med. 2005; 353:2477-90

More than 70% of renal cell carcinomas are clear cell carcinomas. Papillary carcinomas are the second most common renal cell carcinomas. The least common types of renal cell carcinomas are collecting-duct renal cell carcinomas, accounting for less than 1% of all cases.[1]

References

  1. 1.0 1.1 1.2 Cohen HT, McGovern FJ (2005). "Renal-cell carcinoma". N Engl J Med. 353 (23): 2477–90. doi:10.1056/NEJMra043172. PMID 16339096.
  2. Kovacs G, Akhtar M, Beckwith BJ, Bugert P, Cooper CS, Delahunt B; et al. (1997). "The Heidelberg classification of renal cell tumours". J Pathol. 183 (2): 131–3. doi:10.1002/(SICI)1096-9896(199710)183:2<131::AID-PATH931>3.0.CO;2-G. PMID 9390023.


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