Difference between revisions of "Clear cell renal cell carcinoma"

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Clear cell renal cell carcinoma (view source)

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 | Caption    = Clear cell renal cell carcinoma. [[H&E stain]].
 | Synonyms   = conventional renal cell carcinoma
-| Micro      = solid or [[trabecular]] pattern, polygonal cells usually with clear cytoplasm, central nucleus, delicate branching vasculature (chicken wire-like), +/-hyaline bodies
+| 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   = classic, eosinophilic variant
+| Subtypes   =
-| LMDDx      = [[chromophobe renal cell carcinoma]], [[clear cell papillary renal cell carcinoma]], [[Xp11.2 translocation carcinoma]], [[adrenocortical carcinoma]], [[alveolar soft part sarcoma]]
+| LMDDx      = [[chromophobe renal cell carcinoma]], [[clear cell papillary renal cell tumour]], [[Xp11.2 translocation carcinoma]], [[ELOC-mutated renal cell carcinoma]], [[renal cell carcinoma with fibromyomatous stroma]], [[benign clear cell clusters of the kidney]], [[alveolar soft part sarcoma]], [[adrenocortical carcinoma]], [[hemangioblastoma of the kidney]]
-| Stains     = [[Hale's colloidal iron stain]] -ve
+| Stains     = PAS +ve, PAS-D -ve, [[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      = golden/yellow colour, +/-hemorrhagic, +/-cystic, +/-necrosis
+| Gross      = golden/yellow colour, +/-hemorrhagic, +/-cystic, +/-[[necrosis]]
 | Grossing   = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]
+| Staging    = [[kidney cancer staging]]
 | Site       = [[kidney]] - see [[kidney tumours]]
 | Assdx      =
@@ Line 44: / Line 45: @@
 *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>
+**[[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>
@@ Line 67: / Line 68: @@
 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.
@@ Line 72: / Line 74: @@
 *Polygonal cells.
 *Central nucleus.
-*+/-Rhabdoid cells:
+*+/-[[Renal cell carcinoma with rhabdoid morphology|Rhabdoid cells]]:
 **Eccentric nucleus.
-**Abundant eosinophilic cytoplasma.
+**Abundant eosinophilic cytoplasm.
 Notes:
@@ Line 83: / 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:
 *[[Chromophobe renal cell carcinoma]].
-*[[Clear cell papillary renal cell carcinoma]].
+**Chromophobe: "translucent" (NOT quite clear), reticulated, Hale's colloidal iron stain+, CK7+ (cell membrane).
+*[[Clear cell papillary renal cell tumour]].
+*[[ELOC-mutated renal cell carcinoma]].
 *[[Xp11.2 translocation carcinoma]].
-*[[Adrenocortical carcinoma]] (ACC)
+*[[Adrenocortical carcinoma]] (ACC).
 **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]].
+*[[Renal cell carcinoma with fibromyomatous stroma]].
+*[[Hemangioblastoma of the kidney]] - especially in [[von Hippel-Lindau disease]].
+**S-100 +ve, inhibin +ve, PAX-8+ve, EMA -ve, CA9 -ve.
 ===Images===
@@ Line 114: / Line 125: @@
 ==Stains==
+*PAS +ve.
+*PAS-D -ve.<ref>{{cite journal |authors=Kolečková M, Tichý T, Melichar B, Veverková L, Hes O, Kolek V, Kolář Z |title=Metastatic Clear Cell Renal Carcinoma Without Evidence of a Primary Renal Tumour Mimicking Advanced Stage of Malignant Lung Tumour |journal=Klin Onkol |volume=32 |issue=Supplementum1 |pages=154–156 |date=2019 |pmid=31064187 |doi= |url=}}</ref><ref>{{cite journal |authors=Liu S, Liu H, Weng B, Hou S |title=Case report: Tongue metastasis as an initial sign of clear cell renal cell carcinoma and its prognosis |journal=Front Oncol |volume=14 |issue= |pages=1473211 |date=2024 |pmid=39931206 |pmc=11807832 |doi=10.3389/fonc.2024.1473211 |url=}}</ref>
 *Hale's colloidal iron -ve.
 **+ve in [[chromophobe RCC]].
-Note: ''[[Hale's colloidal iron]]'' does not stain iron... it stains hemosiderin.<ref>Latta. 27 January 2009.</ref>
+Note:
+*''[[Hale's colloidal iron]]'' does not stain iron... it stains hemosiderin.<ref>Latta. 27 January 2009.</ref>
-Clear cell vs. chromophobe:
-*Chromophobe: "translucent" (NOT quite clear), reticulated, Hale's colloidal iron stain+, CK7+ (cell membrane).
 ==IHC==
 *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:
@@ Line 136: / Line 149: @@
 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 177: / Line 191: @@
 <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 189: / Line 203: @@
 *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 216: / Line 238: @@
 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 227: / Line 249: @@
 ==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 03:22, 7 March 2025

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