Fumarate hydratase-deficient renal cell carcinoma - Revision history

Michael at 21:03, 30 April 2025

2025-04-30T21:03:42Z

← Older revision		Revision as of 21:03, 30 April 2025
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	==See also==		==See also==
	*[[Vancouver classification]]		*[[Vancouver classification]].
			*[[Sex cord-stromal tumours‎]].

	==References==		==References==

Michael at 16:27, 28 February 2025

2025-02-28T16:27:06Z

← Older revision		Revision as of 16:27, 28 February 2025
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	*Often aggressive - significant cause of mortality.<ref name=pmid24441663>{{Cite journal \| last1 = Chen \| first1 = YB. \| last2 = Brannon \| first2 = AR. \| last3 = Toubaji \| first3 = A. \| last4 = Dudas \| first4 = ME. \| last5 = Won \| first5 = HH. \| last6 = Al-Ahmadie \| first6 = HA. \| last7 = Fine \| first7 = SW. \| last8 = Gopalan \| first8 = A. \| last9 = Frizzell \| first9 = N. \| title = Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cancer: recognition of the syndrome by pathologic features and the utility of detecting aberrant succination by immunohistochemistry. \| journal = Am J Surg Pathol \| volume = 38 \| issue = 5 \| pages = 627-37 \| month = May \| year = 2014 \| doi = 10.1097/PAS.0000000000000163 \| PMID = 24441663 }}</ref>		*Often aggressive - significant cause of mortality.<ref name=pmid24441663>{{Cite journal \| last1 = Chen \| first1 = YB. \| last2 = Brannon \| first2 = AR. \| last3 = Toubaji \| first3 = A. \| last4 = Dudas \| first4 = ME. \| last5 = Won \| first5 = HH. \| last6 = Al-Ahmadie \| first6 = HA. \| last7 = Fine \| first7 = SW. \| last8 = Gopalan \| first8 = A. \| last9 = Frizzell \| first9 = N. \| title = Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cancer: recognition of the syndrome by pathologic features and the utility of detecting aberrant succination by immunohistochemistry. \| journal = Am J Surg Pathol \| volume = 38 \| issue = 5 \| pages = 627-37 \| month = May \| year = 2014 \| doi = 10.1097/PAS.0000000000000163 \| PMID = 24441663 }}</ref>
	*See ''[[hereditary leiomyomatosis and renal cell carcinoma syndrome]]''.		*See ''[[hereditary leiomyomatosis and renal cell carcinoma syndrome]]''.
	*Small tumours may metastasize.		*Small tumours in the context of HLRCC may metastasize.<ref name=pmid17509289>{{cite journal \|authors=Grubb RL, Franks ME, Toro J, Middelton L, Choyke L, Fowler S, Torres-Cabala C, Glenn GM, Choyke P, Merino MJ, Zbar B, Pinto PA, Srinivasan R, Coleman JA, Linehan WM \|title=Hereditary leiomyomatosis and renal cell cancer: a syndrome associated with an aggressive form of inherited renal cancer \|journal=J Urol \|volume=177 \|issue=6 \|pages=2074–9; discussion 2079–80 \|date=June 2007 \|pmid=17509289 \|doi=10.1016/j.juro.2007.01.155 \|url=}}</ref>

	==Microscopic==		==Microscopic==

Michael: /* General */

2025-02-28T16:24:37Z

General

← Older revision		Revision as of 16:24, 28 February 2025
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	*Often aggressive - significant cause of mortality.<ref name=pmid24441663>{{Cite journal \| last1 = Chen \| first1 = YB. \| last2 = Brannon \| first2 = AR. \| last3 = Toubaji \| first3 = A. \| last4 = Dudas \| first4 = ME. \| last5 = Won \| first5 = HH. \| last6 = Al-Ahmadie \| first6 = HA. \| last7 = Fine \| first7 = SW. \| last8 = Gopalan \| first8 = A. \| last9 = Frizzell \| first9 = N. \| title = Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cancer: recognition of the syndrome by pathologic features and the utility of detecting aberrant succination by immunohistochemistry. \| journal = Am J Surg Pathol \| volume = 38 \| issue = 5 \| pages = 627-37 \| month = May \| year = 2014 \| doi = 10.1097/PAS.0000000000000163 \| PMID = 24441663 }}</ref>		*Often aggressive - significant cause of mortality.<ref name=pmid24441663>{{Cite journal \| last1 = Chen \| first1 = YB. \| last2 = Brannon \| first2 = AR. \| last3 = Toubaji \| first3 = A. \| last4 = Dudas \| first4 = ME. \| last5 = Won \| first5 = HH. \| last6 = Al-Ahmadie \| first6 = HA. \| last7 = Fine \| first7 = SW. \| last8 = Gopalan \| first8 = A. \| last9 = Frizzell \| first9 = N. \| title = Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cancer: recognition of the syndrome by pathologic features and the utility of detecting aberrant succination by immunohistochemistry. \| journal = Am J Surg Pathol \| volume = 38 \| issue = 5 \| pages = 627-37 \| month = May \| year = 2014 \| doi = 10.1097/PAS.0000000000000163 \| PMID = 24441663 }}</ref>
	*See ''[[hereditary leiomyomatosis and renal cell carcinoma syndrome]]''.		*See ''[[hereditary leiomyomatosis and renal cell carcinoma syndrome]]''.
			*Small tumours may metastasize.

	==Microscopic==		==Microscopic==

Michael: tweak

2024-03-24T18:35:59Z

tweak

← Older revision		Revision as of 18:35, 24 March 2024
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	\| Micro = cells with large (usually eosinophilic) nucleolus with perinucleolar clearing (may be focal), variable architecture: papillary (classic description) +/-hyaline material within the fibrovascular cores (characteristic), tubulopapillary, tubular, solid, sieve-like pattern/cribriform		\| Micro = cells with large (usually eosinophilic) nucleolus with perinucleolar clearing (may be focal), variable architecture: papillary (classic description) +/-hyaline material within the fibrovascular cores (characteristic), tubulopapillary, tubular, solid, sieve-like pattern/cribriform
	\| Subtypes =		\| Subtypes =
	\| LMDDx = [[papillary renal cell carcinoma]] ~~(type 2)~~, [[tubulocystic carcinoma of the kidney]], [[collecting duct carcinoma]], [[renal medullary carcinoma]]		\| LMDDx = [[papillary renal cell carcinoma]], [[tubulocystic carcinoma of the kidney]], [[collecting duct carcinoma]], [[renal medullary carcinoma]]
	\| Stains =		\| Stains =
	\| IHC = FH -ve, 2SC +ve, CK7 -ve, TFE3 -ve, CK20 -ve, CD10 -ve, UEA-1 -ve		\| IHC = FH -ve, 2SC +ve, CK7 -ve, TFE3 -ve, CK20 -ve, CD10 -ve, UEA-1 -ve
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	DDx:		DDx:
	*[[Papillary renal cell carcinoma]] ~~(type 2)~~.		*[[Papillary renal cell carcinoma]].
	*[[Tubulocystic carcinoma of the kidney]].		*[[Tubulocystic carcinoma of the kidney]].
	*[[Collecting duct carcinoma]].<ref name=pmid16597677>{{Cite journal \| last1 = Pithukpakorn \| first1 = M. \| last2 = Wei \| first2 = MH. \| last3 = Toure \| first3 = O. \| last4 = Steinbach \| first4 = PJ. \| last5 = Glenn \| first5 = GM. \| last6 = Zbar \| first6 = B. \| last7 = Linehan \| first7 = WM. \| last8 = Toro \| first8 = JR. \| title = Fumarate hydratase enzyme activity in lymphoblastoid cells and fibroblasts of individuals in families with hereditary leiomyomatosis and renal cell cancer. \| journal = J Med Genet \| volume = 43 \| issue = 9 \| pages = 755-62 \| month = Sep \| year = 2006 \| doi = 10.1136/jmg.2006.041087 \| PMID = 16597677 }}</ref>		*[[Collecting duct carcinoma]].<ref name=pmid16597677>{{Cite journal \| last1 = Pithukpakorn \| first1 = M. \| last2 = Wei \| first2 = MH. \| last3 = Toure \| first3 = O. \| last4 = Steinbach \| first4 = PJ. \| last5 = Glenn \| first5 = GM. \| last6 = Zbar \| first6 = B. \| last7 = Linehan \| first7 = WM. \| last8 = Toro \| first8 = JR. \| title = Fumarate hydratase enzyme activity in lymphoblastoid cells and fibroblasts of individuals in families with hereditary leiomyomatosis and renal cell cancer. \| journal = J Med Genet \| volume = 43 \| issue = 9 \| pages = 755-62 \| month = Sep \| year = 2006 \| doi = 10.1136/jmg.2006.041087 \| PMID = 16597677 }}</ref>
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	==References==		==References==
	{{Reflist\|1}}		{{Reflist\|2}}

	[[Category:Kidney tumours]]		[[Category:Kidney tumours]]
	[[Category:Diagnosis]]		[[Category:Diagnosis]]

Michael at 18:34, 24 March 2024

2024-03-24T18:34:29Z

← Older revision		Revision as of 18:34, 24 March 2024
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	\| Tx = resection		\| Tx = resection
	}}		}}
	'''Fumarate hydratase-deficient renal cell carcinoma''' (abbreviated '''FH-deficient RCC''') is a formally recognized a distinct entity by the [[WHO]] as of 2013.<ref name=pmid24025519>{{Cite journal \| last1 = Srigley \| first1 = JR. \| last2 = Delahunt \| first2 = B. \| last3 = Eble \| first3 = JN. \| last4 = Egevad \| first4 = L. \| last5 = Epstein \| first5 = JI. \| last6 = Grignon \| first6 = D. \| last7 = Hes \| first7 = O. \| last8 = Moch \| first8 = H. \| last9 = Montironi \| first9 = R. \| title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. \| journal = Am J Surg Pathol \| volume = 37 \| issue = 10 \| pages = 1469-89 \| month = Oct \| year = 2013 \| doi = 10.1097/PAS.0b013e318299f2d1 \| PMID = 24025519 }}</ref>		'''Fumarate hydratase-deficient renal cell carcinoma''' (abbreviated '''FH-deficient RCC''') is a formally recognized a distinct entity by the [[WHO]] as of 2013.

	It was previously known as '''hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma''' (abbreviated '''HLRCC syndrome-associated RCC''').<ref name=pmid26900816>{{Cite journal \| last1 = Trpkov \| first1 = K. \| last2 = Hes \| first2 = O. \| last3 = Agaimy \| first3 = A. \| last4 = Bonert \| first4 = M. \| last5 = Martinek \| first5 = P. \| last6 = Magi-Galluzzi \| first6 = C. \| last7 = Kristiansen \| first7 = G. \| last8 = Lüders \| first8 = C. \| last9 = Nesi \| first9 = G. \| title = Fumarate Hydratase-deficient Renal Cell Carcinoma Is Strongly Correlated With Fumarate Hydratase Mutation and Hereditary Leiomyomatosis and Renal Cell Carcinoma Syndrome. \| journal = Am J Surg Pathol \| volume = \| issue = \| pages = \| month = Feb \| year = 2016 \| doi = 10.1097/PAS.0000000000000617 \| PMID = 26900816 }}</ref>		It was previously known as '''hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma''' (abbreviated '''HLRCC syndrome-associated RCC''').<ref name=pmid24025519>{{Cite journal \| last1 = Srigley \| first1 = JR. \| last2 = Delahunt \| first2 = B. \| last3 = Eble \| first3 = JN. \| last4 = Egevad \| first4 = L. \| last5 = Epstein \| first5 = JI. \| last6 = Grignon \| first6 = D. \| last7 = Hes \| first7 = O. \| last8 = Moch \| first8 = H. \| last9 = Montironi \| first9 = R. \| title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. \| journal = Am J Surg Pathol \| volume = 37 \| issue = 10 \| pages = 1469-89 \| month = Oct \| year = 2013 \| doi = 10.1097/PAS.0b013e318299f2d1 \| PMID = 24025519 }}</ref><ref name=pmid26900816>{{Cite journal \| last1 = Trpkov \| first1 = K. \| last2 = Hes \| first2 = O. \| last3 = Agaimy \| first3 = A. \| last4 = Bonert \| first4 = M. \| last5 = Martinek \| first5 = P. \| last6 = Magi-Galluzzi \| first6 = C. \| last7 = Kristiansen \| first7 = G. \| last8 = Lüders \| first8 = C. \| last9 = Nesi \| first9 = G. \| title = Fumarate Hydratase-deficient Renal Cell Carcinoma Is Strongly Correlated With Fumarate Hydratase Mutation and Hereditary Leiomyomatosis and Renal Cell Carcinoma Syndrome. \| journal = Am J Surg Pathol \| volume = \| issue = \| pages = \| month = Feb \| year = 2016 \| doi = 10.1097/PAS.0000000000000617 \| PMID = 26900816 }}</ref>

	==General==		==General==

Michael at 18:32, 24 March 2024

2024-03-24T18:32:42Z

← Older revision		Revision as of 18:32, 24 March 2024
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	'''Fumarate hydratase-deficient renal cell carcinoma''' (abbreviated '''FH-deficient RCC''') is a formally recognized a distinct entity by the [[WHO]] as of 2013.<ref name=pmid24025519>{{Cite journal \| last1 = Srigley \| first1 = JR. \| last2 = Delahunt \| first2 = B. \| last3 = Eble \| first3 = JN. \| last4 = Egevad \| first4 = L. \| last5 = Epstein \| first5 = JI. \| last6 = Grignon \| first6 = D. \| last7 = Hes \| first7 = O. \| last8 = Moch \| first8 = H. \| last9 = Montironi \| first9 = R. \| title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. \| journal = Am J Surg Pathol \| volume = 37 \| issue = 10 \| pages = 1469-89 \| month = Oct \| year = 2013 \| doi = 10.1097/PAS.0b013e318299f2d1 \| PMID = 24025519 }}</ref>		'''Fumarate hydratase-deficient renal cell carcinoma''' (abbreviated '''FH-deficient RCC''') is a formally recognized a distinct entity by the [[WHO]] as of 2013.<ref name=pmid24025519>{{Cite journal \| last1 = Srigley \| first1 = JR. \| last2 = Delahunt \| first2 = B. \| last3 = Eble \| first3 = JN. \| last4 = Egevad \| first4 = L. \| last5 = Epstein \| first5 = JI. \| last6 = Grignon \| first6 = D. \| last7 = Hes \| first7 = O. \| last8 = Moch \| first8 = H. \| last9 = Montironi \| first9 = R. \| title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. \| journal = Am J Surg Pathol \| volume = 37 \| issue = 10 \| pages = 1469-89 \| month = Oct \| year = 2013 \| doi = 10.1097/PAS.0b013e318299f2d1 \| PMID = 24025519 }}</ref>

	It was previously known as '''hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma''' (abbreviated '''HLRCC syndrome-associated RCC''').		It was previously known as '''hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma''' (abbreviated '''HLRCC syndrome-associated RCC''').<ref name=pmid26900816>{{Cite journal \| last1 = Trpkov \| first1 = K. \| last2 = Hes \| first2 = O. \| last3 = Agaimy \| first3 = A. \| last4 = Bonert \| first4 = M. \| last5 = Martinek \| first5 = P. \| last6 = Magi-Galluzzi \| first6 = C. \| last7 = Kristiansen \| first7 = G. \| last8 = Lüders \| first8 = C. \| last9 = Nesi \| first9 = G. \| title = Fumarate Hydratase-deficient Renal Cell Carcinoma Is Strongly Correlated With Fumarate Hydratase Mutation and Hereditary Leiomyomatosis and Renal Cell Carcinoma Syndrome. \| journal = Am J Surg Pathol \| volume = \| issue = \| pages = \| month = Feb \| year = 2016 \| doi = 10.1097/PAS.0000000000000617 \| PMID = 26900816 }}</ref>

	~~It was previously known as ''HLRCC syndrome-associated RCC'', but clinical criteria for HLRCC syndrome are not met ''or'' the clinical information is not available~~.<ref name=pmid26900816>{{Cite journal \| last1 = Trpkov \| first1 = K. \| last2 = Hes \| first2 = O. \| last3 = Agaimy \| first3 = A. \| last4 = Bonert \| first4 = M. \| last5 = Martinek \| first5 = P. \| last6 = Magi-Galluzzi \| first6 = C. \| last7 = Kristiansen \| first7 = G. \| last8 = Lüders \| first8 = C. \| last9 = Nesi \| first9 = G. \| title = Fumarate Hydratase-deficient Renal Cell Carcinoma Is Strongly Correlated With Fumarate Hydratase Mutation and Hereditary Leiomyomatosis and Renal Cell Carcinoma Syndrome. \| journal = Am J Surg Pathol \| volume = \| issue = \| pages = \| month = Feb \| year = 2016 \| doi = 10.1097/PAS.0000000000000617 \| PMID = 26900816 }}</ref>

	==General==		==General==

Michael: tweak

2024-03-24T18:31:08Z

tweak

← Older revision		Revision as of 18:31, 24 March 2024
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	\| Tx = resection		\| Tx = resection
	}}		}}
	'''~~Hereditary leiomyomatosis and renal cell carcinoma syndrome~~-~~associated~~ renal cell carcinoma''' (abbreviated '''~~HLRCC syndrome~~-~~associated~~ RCC''') is a [[~~cancer\|malignant~~]] ~~epithelial tumour~~ of ~~the [[kidney]] associated with the [[hereditary leiomyomatosis and renal cell carcinoma syndrome]]~~.		'''Fumarate hydratase-deficient renal cell carcinoma''' (abbreviated '''FH-deficient RCC''') is a formally recognized a distinct entity by the [[WHO]] as of 2013.<ref name=pmid24025519>{{Cite journal \| last1 = Srigley \| first1 = JR. \| last2 = Delahunt \| first2 = B. \| last3 = Eble \| first3 = JN. \| last4 = Egevad \| first4 = L. \| last5 = Epstein \| first5 = JI. \| last6 = Grignon \| first6 = D. \| last7 = Hes \| first7 = O. \| last8 = Moch \| first8 = H. \| last9 = Montironi \| first9 = R. \| title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. \| journal = Am J Surg Pathol \| volume = 37 \| issue = 10 \| pages = 1469-89 \| month = Oct \| year = 2013 \| doi = 10.1097/PAS.0b013e318299f2d1 \| PMID = 24025519 }}</ref>

	It ~~is formally recognized a distinct entity by the [[WHO]]~~ as of 2013.<ref name=pmid24025519>{{Cite journal \| last1 = Srigley \| first1 = JR. \| last2 = Delahunt \| first2 = B. \| last3 = Eble \| first3 = JN. \| last4 = Egevad \| first4 = L. \| last5 = Epstein \| first5 = JI. \| last6 = Grignon \| first6 = D. \| last7 = Hes \| first7 = O. \| last8 = Moch \| first8 = H. \| last9 = Montironi \| first9 = R. \| title = The International Society of Urological Pathology (~~ISUP~~) ~~Vancouver Classification of Renal Neoplasia. \| journal = Am J Surg Pathol \| volume = 37 \| issue = 10 \| pages = 1469-89 \| month = Oct \| year = 2013 \| doi = 10.1097/PAS~~.~~0b013e318299f2d1 \| PMID = 24025519 }}</ref>~~		It was previously known as '''hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma''' (abbreviated '''HLRCC syndrome-associated RCC''').

	~~'''Fumarate hydratase-deficient renal cell carcinoma''' (abbreviated '''FH-deficient RCC''') redirects here.~~ It ~~is a term used when pathologic criteria suggest~~ ''HLRCC syndrome-associated RCC'', but clinical criteria for HLRCC syndrome are not met ''or'' the clinical information is not available.<ref name=pmid26900816>{{Cite journal \| last1 = Trpkov \| first1 = K. \| last2 = Hes \| first2 = O. \| last3 = Agaimy \| first3 = A. \| last4 = Bonert \| first4 = M. \| last5 = Martinek \| first5 = P. \| last6 = Magi-Galluzzi \| first6 = C. \| last7 = Kristiansen \| first7 = G. \| last8 = Lüders \| first8 = C. \| last9 = Nesi \| first9 = G. \| title = Fumarate Hydratase-deficient Renal Cell Carcinoma Is Strongly Correlated With Fumarate Hydratase Mutation and Hereditary Leiomyomatosis and Renal Cell Carcinoma Syndrome. \| journal = Am J Surg Pathol \| volume = \| issue = \| pages = \| month = Feb \| year = 2016 \| doi = 10.1097/PAS.0000000000000617 \| PMID = 26900816 }}</ref>		It was previously known as ''HLRCC syndrome-associated RCC'', but clinical criteria for HLRCC syndrome are not met ''or'' the clinical information is not available.<ref name=pmid26900816>{{Cite journal \| last1 = Trpkov \| first1 = K. \| last2 = Hes \| first2 = O. \| last3 = Agaimy \| first3 = A. \| last4 = Bonert \| first4 = M. \| last5 = Martinek \| first5 = P. \| last6 = Magi-Galluzzi \| first6 = C. \| last7 = Kristiansen \| first7 = G. \| last8 = Lüders \| first8 = C. \| last9 = Nesi \| first9 = G. \| title = Fumarate Hydratase-deficient Renal Cell Carcinoma Is Strongly Correlated With Fumarate Hydratase Mutation and Hereditary Leiomyomatosis and Renal Cell Carcinoma Syndrome. \| journal = Am J Surg Pathol \| volume = \| issue = \| pages = \| month = Feb \| year = 2016 \| doi = 10.1097/PAS.0000000000000617 \| PMID = 26900816 }}</ref>

	==General==		==General==

Michael: rename

2024-03-24T18:26:59Z

rename

← Older revision		Revision as of 18:26, 24 March 2024
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	\| Image = Hereditary leiomyomatosis and renal cell carcinoma associated RCC -- high mag.jpg		\| Image = Hereditary leiomyomatosis and renal cell carcinoma associated RCC -- high mag.jpg
	\| Width =		\| Width =
	\| Caption = ~~[[Hereditary leiomyomatosis and renal cell carcinoma syndrome~~-~~associated~~ renal cell carcinoma]] showing the characteristic hyalinized papillary cores. [[H&E stain]]. (WC/Nephron)		\| Caption = FH-deficient renal cell carcinoma showing the characteristic hyalinized papillary cores. [[H&E stain]]. (WC/Nephron)
	\| Synonyms =		\| Synonyms =
	\| Micro = cells with large (usually eosinophilic) nucleolus with perinucleolar clearing (may be focal), variable architecture: papillary (classic description) +/-hyaline material within the fibrovascular cores (characteristic), tubulopapillary, tubular, solid, sieve-like pattern/cribriform		\| Micro = cells with large (usually eosinophilic) nucleolus with perinucleolar clearing (may be focal), variable architecture: papillary (classic description) +/-hyaline material within the fibrovascular cores (characteristic), tubulopapillary, tubular, solid, sieve-like pattern/cribriform

Michael: move

2024-03-24T18:25:26Z

move

← Older revision		Revision as of 18:25, 24 March 2024
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	~~#redirect~~ [[Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma]]		{{ Infobox diagnosis
			\| Name = {{PAGENAME}}
			\| Image = Hereditary leiomyomatosis and renal cell carcinoma associated RCC -- high mag.jpg
			\| Width =
			\| Caption = [[Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma]] showing the characteristic hyalinized papillary cores. [[H&E stain]]. (WC/Nephron)
			\| Synonyms =
			\| Micro = cells with large (usually eosinophilic) nucleolus with perinucleolar clearing (may be focal), variable architecture: papillary (classic description) +/-hyaline material within the fibrovascular cores (characteristic), tubulopapillary, tubular, solid, sieve-like pattern/cribriform
			\| Subtypes =
			\| LMDDx = [[papillary renal cell carcinoma]] (type 2), [[tubulocystic carcinoma of the kidney]], [[collecting duct carcinoma]], [[renal medullary carcinoma]]
			\| Stains =
			\| IHC = FH -ve, 2SC +ve, CK7 -ve, TFE3 -ve, CK20 -ve, CD10 -ve, UEA-1 -ve
			\| EM =
			\| Molecular = FH mutation
			\| IF =
			\| Gross =
			\| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]
			\| Staging = [[kidney cancer staging]]
			\| Site = [[kidney]] - see ''[[kidney tumours]]''
			\| Assdx = [[uterine leiomyoma]]s (women), skin [[leiomyoma]]s
			\| Syndromes = [[hereditary leiomyomatosis and renal cell carcinoma syndrome]]
			\| Clinicalhx = +/-family history of kidney cancer, +/-family history of leiomyomas
			\| Signs = +/-"skin rash" (leiomyomas), signs of renal cancer (+/-hematuria, +/-palpable abdominal mass)
			\| Symptoms =
			\| Prevalence = rare
			\| Bloodwork =
			\| Rads =
			\| Endoscopy =
			\| Prognosis = poor
			\| Other =
			\| ClinDDx = other [[kidney tumours]]
			\| Tx = resection
			}}
			'''Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma''' (abbreviated '''HLRCC syndrome-associated RCC''') is a [[cancer\|malignant]] epithelial tumour of the [[kidney]] associated with the [[hereditary leiomyomatosis and renal cell carcinoma syndrome]].

			It is formally recognized a distinct entity by the [[WHO]] as of 2013.<ref name=pmid24025519>{{Cite journal \| last1 = Srigley \| first1 = JR. \| last2 = Delahunt \| first2 = B. \| last3 = Eble \| first3 = JN. \| last4 = Egevad \| first4 = L. \| last5 = Epstein \| first5 = JI. \| last6 = Grignon \| first6 = D. \| last7 = Hes \| first7 = O. \| last8 = Moch \| first8 = H. \| last9 = Montironi \| first9 = R. \| title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. \| journal = Am J Surg Pathol \| volume = 37 \| issue = 10 \| pages = 1469-89 \| month = Oct \| year = 2013 \| doi = 10.1097/PAS.0b013e318299f2d1 \| PMID = 24025519 }}</ref>

			'''Fumarate hydratase-deficient renal cell carcinoma''' (abbreviated '''FH-deficient RCC''') redirects here. It is a term used when pathologic criteria suggest ''HLRCC syndrome-associated RCC'', but clinical criteria for HLRCC syndrome are not met ''or'' the clinical information is not available.<ref name=pmid26900816>{{Cite journal \| last1 = Trpkov \| first1 = K. \| last2 = Hes \| first2 = O. \| last3 = Agaimy \| first3 = A. \| last4 = Bonert \| first4 = M. \| last5 = Martinek \| first5 = P. \| last6 = Magi-Galluzzi \| first6 = C. \| last7 = Kristiansen \| first7 = G. \| last8 = Lüders \| first8 = C. \| last9 = Nesi \| first9 = G. \| title = Fumarate Hydratase-deficient Renal Cell Carcinoma Is Strongly Correlated With Fumarate Hydratase Mutation and Hereditary Leiomyomatosis and Renal Cell Carcinoma Syndrome. \| journal = Am J Surg Pathol \| volume = \| issue = \| pages = \| month = Feb \| year = 2016 \| doi = 10.1097/PAS.0000000000000617 \| PMID = 26900816 }}</ref>

			==General==
			*Often aggressive - significant cause of mortality.<ref name=pmid24441663>{{Cite journal \| last1 = Chen \| first1 = YB. \| last2 = Brannon \| first2 = AR. \| last3 = Toubaji \| first3 = A. \| last4 = Dudas \| first4 = ME. \| last5 = Won \| first5 = HH. \| last6 = Al-Ahmadie \| first6 = HA. \| last7 = Fine \| first7 = SW. \| last8 = Gopalan \| first8 = A. \| last9 = Frizzell \| first9 = N. \| title = Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cancer: recognition of the syndrome by pathologic features and the utility of detecting aberrant succination by immunohistochemistry. \| journal = Am J Surg Pathol \| volume = 38 \| issue = 5 \| pages = 627-37 \| month = May \| year = 2014 \| doi = 10.1097/PAS.0000000000000163 \| PMID = 24441663 }}</ref>
			*See ''[[hereditary leiomyomatosis and renal cell carcinoma syndrome]]''.

			==Microscopic==
			Features - renal cell carcinoma:<ref name=pmid24441663>{{Cite journal \| last1 = Chen \| first1 = YB. \| last2 = Brannon \| first2 = AR. \| last3 = Toubaji \| first3 = A. \| last4 = Dudas \| first4 = ME. \| last5 = Won \| first5 = HH. \| last6 = Al-Ahmadie \| first6 = HA. \| last7 = Fine \| first7 = SW. \| last8 = Gopalan \| first8 = A. \| last9 = Frizzell \| first9 = N. \| title = Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cancer: recognition of the syndrome by pathologic features and the utility of detecting aberrant succination by immunohistochemistry. \| journal = Am J Surg Pathol \| volume = 38 \| issue = 5 \| pages = 627-37 \| month = May \| year = 2014 \| doi = 10.1097/PAS.0000000000000163 \| PMID = 24441663 }}</ref>
			*Cells with:
			**Large, (usually) eosinophilic, nucleolus with perinucleolar clearing - proposed hallmark - '''important'''.
			***May be focal.
			**Abundant or moderate (eosinophilic/partially clear) cytoplasm.
			*Variable architecture:
			**Papillary - classic description.
			***Hyaline material within the fibrovascular cores - '''characteristic'''.
			**Tubulopapillary.
			**Tubular.
			**Solid.
			**[[Sieve-like pattern]]/[[cribriform]].

			Notes:
			*Not common: [[psammoma bodies]], foamy macrophages.<ref name=pmid11248088>{{Cite journal \| last1 = Launonen \| first1 = V. \| last2 = Vierimaa \| first2 = O. \| last3 = Kiuru \| first3 = M. \| last4 = Isola \| first4 = J. \| last5 = Roth \| first5 = S. \| last6 = Pukkala \| first6 = E. \| last7 = Sistonen \| first7 = P. \| last8 = Herva \| first8 = R. \| last9 = Aaltonen \| first9 = LA. \| title = Inherited susceptibility to uterine leiomyomas and renal cell cancer. \| journal = Proc Natl Acad Sci U S A \| volume = 98 \| issue = 6 \| pages = 3387-92 \| month = Mar \| year = 2001 \| doi = 10.1073/pnas.051633798 \| PMID = 11248088 }}</ref>

			DDx:
			*[[Papillary renal cell carcinoma]] (type 2).
			*[[Tubulocystic carcinoma of the kidney]].
			*[[Collecting duct carcinoma]].<ref name=pmid16597677>{{Cite journal \| last1 = Pithukpakorn \| first1 = M. \| last2 = Wei \| first2 = MH. \| last3 = Toure \| first3 = O. \| last4 = Steinbach \| first4 = PJ. \| last5 = Glenn \| first5 = GM. \| last6 = Zbar \| first6 = B. \| last7 = Linehan \| first7 = WM. \| last8 = Toro \| first8 = JR. \| title = Fumarate hydratase enzyme activity in lymphoblastoid cells and fibroblasts of individuals in families with hereditary leiomyomatosis and renal cell cancer. \| journal = J Med Genet \| volume = 43 \| issue = 9 \| pages = 755-62 \| month = Sep \| year = 2006 \| doi = 10.1136/jmg.2006.041087 \| PMID = 16597677 }}</ref>
			*[[Renal medullary carcinoma]] - cells also have a [[prominent nucleoli\|prominent nucleolus]].

			===Images===
			====RCC====
			<gallery>
			Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC -- low mag.jpg \| HLRCC - low mag. (WC)
			Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC -- intermed mag.jpg \| HLRCC - intermed mag. (WC)
			Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC -- high mag.jpg \| HLRCC - high mag. (WC)
			Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC - alt -- high mag.jpg \| HLRCC - high mag. (WC)
			Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC -- very high mag.jpg \| HLRCC - very high mag. (WC)
			Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC - alt -- very high mag.jpg \| HLRCC - very high mag. (WC)
			</gallery>
			<gallery>
			Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC - TC-like -- low mag.jpg \| HLRCC - [[tubulocystic carcinoma of the kidney\|TC-like]] - low mag.
			Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC - TC-like -- intermed mag.jpg \| HLRCC - TC-like - intermed mag.
			Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC - TC-like -- high mag.jpg \| HLRCC - TC-like - high mag.
			Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC - TC-like -- very high mag.jpg \| HLRCC - TC-like - very high mag.
			</gallery>

			====www====
			*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC30663/figure/F3/ RCC in HLRCC (nih.gov)].<ref name=pmid11248088/>

			==IHC==
			*Fumarate hydratase (FH) -ve.
			*2SC +ve -- cytoplasmic,<ref name=pmid24309325 >{{Cite journal \| last1 = Reyes \| first1 = C. \| last2 = Karamurzin \| first2 = Y. \| last3 = Frizzell \| first3 = N. \| last4 = Garg \| first4 = K. \| last5 = Nonaka \| first5 = D. \| last6 = Chen \| first6 = YB. \| last7 = Soslow \| first7 = RA. \| title = Uterine smooth muscle tumors with features suggesting fumarate hydratase aberration: detailed morphologic analysis and correlation with S-(2-succino)-cysteine immunohistochemistry. \| journal = Mod Pathol \| volume = 27 \| issue = 7 \| pages = 1020-7 \| month = Jul \| year = 2014 \| doi = 10.1038/modpathol.2013.215 \| PMID = 24309325 }}</ref> cytoplasmic and nuclear.<ref name=pmid24441663>{{Cite journal \| last1 = Chen \| first1 = YB. \| last2 = Brannon \| first2 = AR. \| last3 = Toubaji \| first3 = A. \| last4 = Dudas \| first4 = ME. \| last5 = Won \| first5 = HH. \| last6 = Al-Ahmadie \| first6 = HA. \| last7 = Fine \| first7 = SW. \| last8 = Gopalan \| first8 = A. \| last9 = Frizzell \| first9 = N. \| title = Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cancer: recognition of the syndrome by pathologic features and the utility of detecting aberrant succination by immunohistochemistry. \| journal = Am J Surg Pathol \| volume = 38 \| issue = 5 \| pages = 627-37 \| month = May \| year = 2014 \| doi = 10.1097/PAS.0000000000000163 \| PMID = 24441663 }}</ref>
			**2SC = S-(2-succino)-cysteine.

			Others:
			*CK7 -ve (0 +ve/38 cases<ref name=pmid17895761/>).
			*CD10 -ve.
			**May be positive in [[clear cell RCC]]-like areas.
			*CK20 -ve (0 +ve/38 cases<ref name=pmid17895761/>).
			*UEA-1 -ve.<ref name=pmid17895761>{{Cite journal \| last1 = Merino \| first1 = MJ. \| last2 = Torres-Cabala \| first2 = C. \| last3 = Pinto \| first3 = P. \| last4 = Linehan \| first4 = WM. \| title = The morphologic spectrum of kidney tumors in hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome. \| journal = Am J Surg Pathol \| volume = 31 \| issue = 10 \| pages = 1578-85 \| month = Oct \| year = 2007 \| doi = 10.1097/PAS.0b013e31804375b8 \| PMID = 17895761 }}</ref>
			**[[Collecting duct carcinoma]] +ve.
			*TFE3 -ve (0 +ve/38 cases<ref name=pmid17895761/>).
			*CK34betaE12 -ve (0 +ve/38 cases<ref name=pmid17895761/>).

			==See also==
			*[[Vancouver classification]]

			==References==
			{{Reflist\|1}}

			[[Category:Kidney tumours]]
			[[Category:Diagnosis]]

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2016-03-18T12:24:56Z

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