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

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#redirect [[Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma]]