Difference between revisions of "Clear cell renal cell carcinoma"

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Clear cell renal cell carcinoma (view source)

Revision as of 16:14, 22 January 2024

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benign clear cell clusters

Michael

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 {{ Infobox diagnosis
 | Name       = {{PAGENAME}}
-| Image      = Clear cell renal cell carcinoma -- high mag.jpg
+| Image      = Clear cell renal cell carcinoma - 2 -- high mag.jpg
 | Width      =
 | Caption    = Clear cell renal cell carcinoma. [[H&E stain]].
-| Micro      = solid or [[trabecular]] pattern, polygonal cells usually with clear cytoplasm, central nucleus, delicate branching vasculature (chicken wire-like), +/-hyaline bodies
+| Synonyms   = conventional renal cell carcinoma
-| Subtypes   = classic, eosinophilic variant
+| Micro      = solid or [[trabecular]] pattern, polygonal cells usually with clear cytoplasm (may be eosinophilic - esp. in high grade tumours), central nucleus, delicate branching vasculature (chicken wire-like), +/-hyaline bodies
+| Subtypes   =
 | LMDDx      = [[chromophobe renal cell carcinoma]], [[clear cell papillary renal cell carcinoma]], [[Xp11.2 translocation carcinoma]], [[adrenocortical carcinoma]], [[alveolar soft part sarcoma]]
 | Stains     = [[Hale's colloidal iron stain]] -ve
-| IHC        = CK7 -ve, CK20 -ve, TFE3 -ve
+| IHC        = [[CA9]] +ve, CK7 -ve, CK20 -ve, TFE3 -ve, PAX8 +ve, vimentin +ve
 | EM         =
 | Molecular  =
 | IF         =
-| Gross      =
+| Gross      = golden/yellow colour, +/-hemorrhagic, +/-cystic, +/-[[necrosis]]
-| Grossing   =
+| Grossing   = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]
+| Staging    = [[kidney cancer staging]]
 | Site       = [[kidney]] - see [[kidney tumours]]
 | Assdx      =
@@ Line 27: / Line 29: @@
 | Other      =
 | ClinDDx    = other [[renal tumours]]
+| Tx         = surgery, targeted therapies, other drugs
 }}
-'''Clear cell renal cell carcinoma''', abbreviated '''CRCC''' or '''CCRCC''', is the most common type of [[renal cell carcinoma]].
+'''Clear cell renal cell carcinoma''', abbreviated '''CCRCC''', is the most common type of [[renal cell carcinoma]].
+It is also known as '''conventional renal cell carcinoma''' (abbreviated '''CRCC''').
 ==General==
@@ Line 35: / Line 40: @@
 *Familial clear cell renal cell carcinoma<ref name=pmid23972705>{{Cite journal  | last1 = Keefe | first1 = SM. | last2 = Nathanson | first2 = KL. | last3 = Rathmell | first3 = WK. | title = The molecular biology of renal cell carcinoma. | journal = Semin Oncol | volume = 40 | issue = 4 | pages = 421-8 | month = Aug | year = 2013 | doi = 10.1053/j.seminoncol.2013.05.006 | PMID = 23972705 }}</ref> - usually VHL gene mutation.<ref name=pmid17205537>{{Cite journal  | last1 = Foster | first1 = RE. | last2 = Abdulrahman | first2 = M. | last3 = Morris | first3 = MR. | last4 = Prigmore | first4 = E. | last5 = Gribble | first5 = S. | last6 = Ng | first6 = B. | last7 = Gentle | first7 = D. | last8 = Ready | first8 = S. | last9 = Weston | first9 = PM. | title = Characterization of a 3;6 translocation associated with renal cell carcinoma. | journal = Genes Chromosomes Cancer | volume = 46 | issue = 4 | pages = 311-7 | month = Apr | year = 2007 | doi = 10.1002/gcc.20403 | PMID = 17205537 }}</ref>
 **VHL gene not mutated in all cases.<ref name=pmid10807693>{{Cite journal  | last1 = Woodward | first1 = ER. | last2 = Clifford | first2 = SC. | last3 = Astuti | first3 = D. | last4 = Affara | first4 = NA. | last5 = Maher | first5 = ER. | title = Familial clear cell renal cell carcinoma (FCRC): clinical features and mutation analysis of the VHL, MET, and CUL2 candidate genes. | journal = J Med Genet | volume = 37 | issue = 5 | pages = 348-53 | month = May | year = 2000 | doi =  | PMID = 10807693 }}</ref>
+Treatment:
+*Surgery.
+*Various drugs.
+*Targeted therapies.
+**[[Tyrosine kinase inhibitors]] (e.g. [[sunitinib]], sorafenib).<ref name=pmid25467013>{{Cite journal  | last1 = Elaidi | first1 = R. | last2 = Harbaoui | first2 = A. | last3 = Beuselinck | first3 = B. | last4 = Eymard | first4 = JC. | last5 = Bamias | first5 = A. | last6 = De Guillebon | first6 = E. | last7 = Porta | first7 = C. | last8 = Vano | first8 = Y. | last9 = Linassier | first9 = C. | title = Outcomes from second-line therapy in long-term responders to first-line tyrosine kinase inhibitor in clear-cell metastatic renal cell carcinoma. | journal = Ann Oncol | volume = 26 | issue = 2 | pages = 378-85 | month = Feb | year = 2015 | doi = 10.1093/annonc/mdu552 | PMID = 25467013 }}</ref><ref name=pmid21484781>{{Cite journal  | last1 = Verma | first1 = J. | last2 = Jonasch | first2 = E. | last3 = Allen | first3 = P. | last4 = Tannir | first4 = N. | last5 = Mahajan | first5 = A. | title = Impact of tyrosine kinase inhibitors on the incidence of brain metastasis in metastatic renal cell carcinoma. | journal = Cancer | volume = 117 | issue = 21 | pages = 4958-65 | month = Nov | year = 2011 | doi = 10.1002/cncr.26138 | PMID = 21484781 }}</ref>
+**Anti-VEGF antibodies (e.g. bevacizumab).<ref name=pmid21330923>{{Cite journal  | last1 = Singer | first1 = EA. | last2 = Gupta | first2 = GN. | last3 = Srinivasan | first3 = R. | title = Update on targeted therapies for clear cell renal cell carcinoma. | journal = Curr Opin Oncol | volume = 23 | issue = 3 | pages = 283-9 | month = May | year = 2011 | doi = 10.1097/CCO.0b013e32834479c0 | PMID = 21330923 }}</ref>
 ==Gross==
-*Gold/yellow.
+Features:
+*Golden/yellow colour.
 *+/-Haemorrhage (common).
 *+/-Necrosis (common in large tumours).
 *+/-Calcification.
 *+/-Cysts.
+*Usually unifocal. ‡
+Note:
+*‡ Approximately 4% of CCRCCs are multifocal (based on a set of 5378 patients).<ref name=pmid22502873>{{Cite journal  | last1 = Siracusano | first1 = S. | last2 = Novara | first2 = G. | last3 = Antonelli | first3 = A. | last4 = Artibani | first4 = W. | last5 = Bertini | first5 = R. | last6 = Carini | first6 = M. | last7 = Carmignani | first7 = G. | last8 = Ciciliato | first8 = S. | last9 = Cunico | first9 = SC. | title = Prognostic role of tumour multifocality in renal cell carcinoma. | journal = BJU Int | volume = 110 | issue = 11 Pt B | pages = E443-8 | month = Dec | year = 2012 | doi = 10.1111/j.1464-410X.2012.11121.x | PMID = 22502873 }}</ref>
 ===Image===
@@ Line 50: / Line 67: @@
 ==Microscopic==
 Features:<ref name=Ref_PBoD1017-8>{{Ref PBoD|1017-8}}</ref>
+*Clear cytoplasm.
+**May have eosinophilic cytoplasm (in high-grade tumours) - usually focal.
+*Delicate branching vasculature - '''key feature'''.
+**Often called "chicken wire-like" vasculature.
 *Solid or [[trabecular]] pattern.
 *Polygonal cells.
-*Clear cytoplasm.
 *Central nucleus.
-*Delicate branching vasculature.
+*+/-[[Renal cell carcinoma with rhabdoid morphology|Rhabdoid cells]]:
-**Often called "chicken wire-like" vasculature.
-*+/-Rhabdoid cells:
 **Eccentric nucleus.
-**Abundant eosinophilic cytoplasma.
+**Abundant eosinophilic cytoplasm.
 Notes:
@@ Line 67: / Line 85: @@
 **Not common in papillary RCC.
 *Clear cytoplasm - due to lipid content.
+*[[Necrosis]] is independent predictor of outcome - if present in a significant quantity:<ref name=pmid25474516>{{Cite journal  | last1 = Renshaw | first1 = AA. | last2 = Cheville | first2 = JC. | title = Quantitative tumour necrosis is an independent predictor of overall survival in clear cell renal cell carcinoma. | journal = Pathology | volume = 47 | issue = 1 | pages = 34-7 | month = Jan | year = 2015 | doi = 10.1097/PAT.0000000000000193 | PMID = 25474516 }}</ref>
+**>30% is significant for pT3a tumours.
+**>20% is significant for pT1-2 pN0 tumours.
 DDx:
@@ Line 75: / Line 96: @@
 **EMA -ve, CKs mostly -ve, inhibin +ve (neg. in RCC).<ref name=Ref_GUP285>{{Ref GUP|285}}</ref>
 *[[Alveolar soft part sarcoma]].
-*[[Adrenal gland]], normal.
+*[[Adrenal gland]], normal cortex - wispy, vacuolated.
 *[[Epithelioid angiomyolipoma]].
+*[[Benign clear cell clusters of the kidney]].
 ===Images===
+====Case 1====
 <gallery>
-Image:Clear_cell_renal_cell_carcinoma_intermed_mag.jpg | CRCC - intermed. mag. (WC/Nephron)
+Image: Clear cell renal cell carcinoma - 2 -- intermed mag.jpg | CCRCC - intermed. mag. (WC)
-Image:Clear_cell_renal_cell_carcinoma_high_mag.jpg | CRCC - high mag. (WC/Nephron)
+Image: Clear cell renal cell carcinoma - 2 -- high mag.jpg | CCRCC - high mag. (WC)
+Image: Clear cell renal cell carcinoma - 2 -- very high mag.jpg | CCRCC - very high mag. (WC)
 </gallery>
+====Case 2====
 <gallery>
 Image: Clear cell renal cell carcinoma -- intermed mag.jpg | CCRCC - intermed. mag. (WC)
 Image: Clear cell renal cell carcinoma -- high mag.jpg | CCRCC - high mag. (WC)
 Image: Clear cell renal cell carcinoma -- very high mag.jpg | CCRCC - very high mag. (WC)
+</gallery>
+====Case 3====
+<gallery>
+Image:Clear_cell_renal_cell_carcinoma_intermed_mag.jpg | CRCC - intermed. mag. (WC/Nephron)
+Image:Clear_cell_renal_cell_carcinoma_high_mag.jpg | CRCC - high mag. (WC/Nephron)
 </gallery>
@@ Line 101: / Line 131: @@
 *CD10 +ve.
 *Vimentin +ve.
 *CK7 -ve.
+**Strong CK7 staining is more in keeping with [[clear cell papillary renal cell carcinoma]]<ref name=pmid26457355>{{Cite journal  | last1 = Williamson | first1 = SR. | last2 = Gupta | first2 = NS. | last3 = Eble | first3 = JN. | last4 = Rogers | first4 = CG. | last5 = Michalowski | first5 = S. | last6 = Zhang | first6 = S. | last7 = Wang | first7 = M. | last8 = Grignon | first8 = DJ. | last9 = Cheng | first9 = L. | title = Clear Cell Renal Cell Carcinoma With Borderline Features of Clear Cell Papillary Renal Cell Carcinoma: Combined Morphologic, Immunohistochemical, and Cytogenetic Analysis. | journal = Am J Surg Pathol | volume = 39 | issue = 11 | pages = 1502-10 | month = Nov | year = 2015 | doi = 10.1097/PAS.0000000000000514 | PMID = 26457355 }}</ref> or the evolving entity of [[TCEB1-mutated renal cell carcinoma]].
 *CK20 -ve.
+*Glut-1 >85% of CCRCC (membranous pattern).<ref name=pmid17638658>{{Cite journal  | last1 = Ozcan | first1 = A. | last2 = Shen | first2 = SS. | last3 = Zhai | first3 = QJ. | last4 = Truong | first4 = LD. | title = Expression of GLUT1 in primary renal tumors: morphologic and biologic implications. | journal = Am J Clin Pathol | volume = 128 | issue = 2 | pages = 245-54 | month = Aug | year = 2007 | doi = 10.1309/HV6NJVRQKK4QHM9F | PMID = 17638658 }}</ref>
+*E-cadherin +ve - usually weak, often -ve in higher stage tumours.<ref name=pmid23939157>{{Cite journal  | last1 = Hu | first1 = H. | last2 = Chen | first2 = JW. | last3 = Xu | first3 = KX. | last4 = Wang | first4 = D. | last5 = Wang | first5 = Y. | last6 = Wang | first6 = GW. | last7 = Zhang | first7 = SY. | last8 = Wang | first8 = XF. | title = [Expressions of CMTM8 and E-cadherin in primary and metastatic clear cell renal cell carcinoma]. | journal = Beijing Da Xue Xue Bao | volume = 45 | issue = 4 | pages = 537-41 | month = Aug | year = 2013 | doi =  | PMID = 23939157 }}</ref>
+*CAIX (also CA9) +ve.
+A panel to confirm the diagnosis:
+*CK7 (-ve), CD10 (+ve), PAX8 (+ve), vimentin (+ve).
 ===CCRCC versus [[chromophobe RCC]]===
 ISUP recommends:<ref name=pmid25025364 >{{cite journal |author=Amin MB, Epstein JI, Ulbright TM, ''et al.'' |title=Best practices recommendations in the application of immunohistochemistry in urologic pathology: report from the international society of urological pathology consensus conference |journal=Am. J. Surg. Pathol. |volume=38 |issue=8 |pages=1017–22 |year=2014 |month=August |pmid=25025364 |doi=10.1097/PAS.0000000000000254 |url=}}</ref>
 *CD117 -ve (+ve in ChRCC).
-*CAIX (carbonic anhydrase CA IX) +ve (membranous).<ref name=pmid21677535>{{cite journal |author=Al-Ahmadie HA, Alden D, Fine SW, ''et al.'' |title=Role of immunohistochemistry in the evaluation of needle core biopsies in adult renal cortical tumors: an ex vivo study |journal=Am. J. Surg. Pathol. |volume=35 |issue=7 |pages=949–61 |year=2011 |month=July |pmid=21677535 |doi=10.1097/PAS.0b013e31821e25cd |url=}}</ref>
+**Aguilar-León ''et al.'' found ~8% of CCRCC are CD117 +ve.<ref name=pmid25643775>{{Cite journal  | last1 = Aguilar-León | first1 = D. | last2 = Del Ángel-Millán | first2 = G. | last3 = Zepeda-Quiroz | first3 = I. | last4 = Soria-Céspedes | first4 = D. | title = [Immunohistochemical Expression of CD117 (c-kit) in 75 Renal Cell Tumors]. | journal = Gac Med Mex | volume = 150 Suppl 2 | issue =  | pages = 156-60 | month = Dec | year = 2014 | doi =  | PMID = 25643775 }}</ref>
-*CK7 -ve (+ve in ChRCC).
+**Liu ''et al.'' found ~80% of ChRCC are CD117 +ve.<ref name=pmid17683191/>
+*CA9 ([[carbonic anhydrase 9]]) +ve (membranous).<ref name=pmid21677535>{{cite journal |author=Al-Ahmadie HA, Alden D, Fine SW, ''et al.'' |title=Role of immunohistochemistry in the evaluation of needle core biopsies in adult renal cortical tumors: an ex vivo study |journal=Am. J. Surg. Pathol. |volume=35 |issue=7 |pages=949–61 |year=2011 |month=July |pmid=21677535 |doi=10.1097/PAS.0b013e31821e25cd |url=}}</ref>
+*[[CK7]] -ve (+ve in ChRCC).
-Another set of stains:<ref name=pmid17683191>{{Cite journal  | last1 = Liu | first1 = L. | last2 = Qian | first2 = J. | last3 = Singh | first3 = H. | last4 = Meiers | first4 = I. | last5 = Zhou | first5 = X. | last6 = Bostwick | first6 = DG. | title = Immunohistochemical analysis of chromophobe renal cell carcinoma, renal oncocytoma, and clear cell carcinoma: an optimal and practical panel for differential diagnosis. | journal = Arch Pathol Lab Med | volume = 131 | issue = 8 | pages = 1290-7 | month = Aug | year = 2007 | doi = 10.1043/1543-2165(2007)131[1290:IAOCRC]2.0.CO;2 | PMID = 17683191 }}</ref>
+May also be useful:
 *Vimentin +ve.
-**-ve in ChRCC.
+**-ve in ChRCC.<ref name=pmid17683191>{{Cite journal  | last1 = Liu | first1 = L. | last2 = Qian | first2 = J. | last3 = Singh | first3 = H. | last4 = Meiers | first4 = I. | last5 = Zhou | first5 = X. | last6 = Bostwick | first6 = DG. | title = Immunohistochemical analysis of chromophobe renal cell carcinoma, renal oncocytoma, and clear cell carcinoma: an optimal and practical panel for differential diagnosis. | journal = Arch Pathol Lab Med | volume = 131 | issue = 8 | pages = 1290-7 | month = Aug | year = 2007 | doi = 10.1043/1543-2165(2007)131[1290:IAOCRC]2.0.CO;2 | PMID = 17683191 }}</ref>
-*CD117 -ve.
-**+ve ~80% of ChRCC.
 ===CCRCC versus [[CCPRCC]]===
 [[ISUP]] recommends:<ref name=pmid25025364/>
-*CAIX +ve (strong membranous).
+*CA9 +ve (strong membranous).
 *CK7 -ve.
+**Usually +ve in [[clear cell papillary renal cell carcinoma]].
 *AMACR -ve.
@@ Line 145: / Line 183: @@
 </pre>
+====MDA form====
 <pre>
 Kidney Mass, Left, Core Biopsy:
-- CLEAR CELL RENAL CELL CARCINOMA, preliminary nucleolar grade 2 (see comment)
+- CLEAR CELL RENAL CELL CARCINOMA, preliminary ISUP nucleolar grade 2 (see comment).
-- No renal parenchyma identified
+- No renal parenchyma identified.
 Comment:
@@ Line 159: / Line 198: @@
 *The surgeon wants the diagnosis and margin status; thus, these are included in the diagnosis line with the tumour stage.
+<pre>
+Left Kidney, Radical Nephrectomy:
+     - CLEAR CELL RENAL CELL CARCINOMA.
+     -- Margins clear.
+     -- Please see synoptic report.
+</pre>
+====Block letters====
 <pre>
 KIDNEY, LEFT, NEPHRECTOMY:
@@ Line 186: / Line 233: @@
 The sections show a tumour with a chicken wire-like vasculature composed of clear cells with basally stratified nuclei. Hemosiderin-laden macrophages are present. Necrosis is present. Abundant hyaline globules are present.
-There is no perinuclear clearing.  The cytoplasm is not whispy.  Collections of macrophages are not identified within the stroma of the tumour.  Papillae are not evident.
+There is no perinuclear clearing.  The cytoplasm is not wispy. Papillae are not evident.
 Rare, small nucleoli are visible with the 10x objective.  The tumour nuclei are approximately 15 micrometers.
@@ Line 197: / Line 244: @@
 ==See also==
 *[[Kidney tumours]].
+*[[TCEB1-mutated renal cell carcinoma]].
+*[[Renal cell carcinoma with rhabdoid morphology]].
 ==References==

Difference between revisions of "Clear cell renal cell carcinoma"

Clear cell renal cell carcinoma (view source)

Revision as of 16:14, 22 January 2024

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