HAEM5:Mycosis fungoides - Revision history

Bailey.Glen at 17:22, 3 July 2025

2025-07-03T17:22:22Z

← Older revision		Revision as of 12:22, 3 July 2025
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	\|}		\|}

	~~==WHO Essential and Desirable Genetic Diagnostic Criteria==~~
	<span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span>
	~~{\| class="wikitable"~~
	\|+
	\|WHO Essential Criteria (Genetics)*
	\|
	\|-
	\|WHO Desirable Criteria (Genetics)*
	\|
	\|-
	~~\|Other Classification~~
	\|
	\|}
	<nowiki>*</nowiki>Note: These are only the genetic/genomic criteria. Additional diagnostic criteria can be found in the [https://tumourclassification.iarc.who.int/home <u>WHO Classification of Tumours</u>].
	==Related Terminology==		==Related Terminology==

Bailey.Glen at 21:41, 25 May 2025

2025-05-25T21:41:51Z

← Older revision		Revision as of 16:41, 25 May 2025
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	{\| class="wikitable sortable"		{\| class="wikitable sortable"
	\|-		\|-
	!Chr #!!~~'''~~Gain, Loss, Amp, LOH~~'''~~!!~~'''~~Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]~~'''~~!!~~'''~~Relevant Gene(s)~~'''~~		!Chr #!!Gain, Loss, Amp, LOH!!Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]!!Relevant Gene(s)
	!~~'''~~Diagnostic, Prognostic, and Therapeutic Significance - D, P, T~~'''~~		!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
	!~~'''~~Established Clinical Significance Per Guidelines - Yes or No (Source)~~'''~~		!Established Clinical Significance Per Guidelines - Yes or No (Source)
	!~~'''~~Clinical Relevance Details/Other Notes~~'''~~		!Clinical Relevance Details/Other Notes
	\|-		\|-
	\|<span class="blue-text">EXAMPLE:</span>		\|<span class="blue-text">EXAMPLE:</span>
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	!Chromosomal Pattern		!Chromosomal Pattern
	!Molecular Pathogenesis		!Molecular Pathogenesis
	!~~'''~~Prevalence -~~'''~~		!Prevalence -
	~~'''~~Common >20%, Recurrent 5-20% or Rare <5% (Disease)~~'''~~		Common >20%, Recurrent 5-20% or Rare <5% (Disease)
	!~~'''~~Diagnostic, Prognostic, and Therapeutic Significance - D, P, T~~'''~~		!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
	!~~'''~~Established Clinical Significance Per Guidelines - Yes or No (Source)~~'''~~		!Established Clinical Significance Per Guidelines - Yes or No (Source)
	!~~'''~~Clinical Relevance Details/Other Notes~~'''~~		!Clinical Relevance Details/Other Notes
	\|-		\|-
	\|<span class="blue-text">EXAMPLE:</span>		\|<span class="blue-text">EXAMPLE:</span>
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	{\| class="wikitable sortable"		{\| class="wikitable sortable"
	\|-		\|-
	!Gene!!~~'''~~Genetic Alteration~~'''~~!!~~'''~~Tumor Suppressor Gene, Oncogene, Other~~'''~~!!~~'''~~Prevalence -~~'''~~		!Gene!!Genetic Alteration!!Tumor Suppressor Gene, Oncogene, Other!!Prevalence -
	~~'''~~Common >20%, Recurrent 5-20% or Rare <5% (Disease)~~'''~~		Common >20%, Recurrent 5-20% or Rare <5% (Disease)
	!~~'''~~Diagnostic, Prognostic, and Therapeutic Significance - D, P, T ~~'''~~		!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
	!~~'''~~Established Clinical Significance Per Guidelines - Yes or No (Source)~~'''~~		!Established Clinical Significance Per Guidelines - Yes or No (Source)
	!~~'''~~Clinical Relevance Details/Other Notes~~'''~~		!Clinical Relevance Details/Other Notes
	\|-		\|-
	\|<span class="blue-text">EXAMPLE:</span>''EGFR''		\|<span class="blue-text">EXAMPLE:</span>''EGFR''

Bailey.Glen at 20:18, 25 May 2025

2025-05-25T20:18:10Z

← Older revision		Revision as of 15:18, 25 May 2025
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	<nowiki>*</nowiki>Note: These are only the genetic/genomic criteria. Additional diagnostic criteria can be found in the [https://tumourclassification.iarc.who.int/home <u>WHO Classification of Tumours</u>].		<nowiki>*</nowiki>Note: These are only the genetic/genomic criteria. Additional diagnostic criteria can be found in the [https://tumourclassification.iarc.who.int/home <u>WHO Classification of Tumours</u>].
	==Related Terminology==		==Related Terminology==
	~~<span style="color:#0070C0">(''Instructions: The table will have the related terminology from the WHO <u>autocompleted</u>.)''</span>~~
	{\| class="wikitable"		{\| class="wikitable"
	\|+		\|+
	\|Acceptable		\|Acceptable
	\|		\|N/A
	\|-		\|-
	\|Not Recommended		\|Not Recommended
	\|		\|Classic mycosis fungoides (type Alibert–Bazin); Woringer–Kolopp disease
	\|}		\|}

Bailey.Glen at 17:40, 24 March 2025

2025-03-24T17:40:51Z

← Older revision		Revision as of 12:40, 24 March 2025
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	{{DISPLAYTITLE:Mycosis fungoides}}		{{DISPLAYTITLE:Mycosis fungoides}}

	[[HAEM5:Table_of_Contents\|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]		[[HAEM5:Table_of_Contents\|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]

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	Jane Scribner, MD and Daynna J. Wolff, PhD		Jane Scribner, MD and Daynna J. Wolff, PhD

	~~__TOC__~~

	==WHO Classification of Disease==		==WHO Classification of Disease==

Bailey.Glen: Removed old template contents

2025-02-11T21:58:50Z

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Bailey.Glen at 18:53, 10 February 2025

2025-02-10T18:53:22Z

Show changes

Bailey.Glen at 18:25, 10 February 2025

2025-02-10T18:25:18Z

← Older revision		Revision as of 13:25, 10 February 2025
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	<blockquote class='blockedit'>{{Box-round\|title=v4:Clinical Features\|The content below was from the old template. Please incorporate above.}}		<blockquote class='blockedit'>{{Box-round\|title=v4:Clinical Features\|The content below was from the old template. Please incorporate above.}}</blockquote>

	*Pruritic, erythematous or poikilodermatous and atrophic skin lesions with a fine scale		*Pruritic, erythematous or poikilodermatous and atrophic skin lesions with a fine scale
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	*May have symptoms and/or diagnosis of other inflammatory skin diseases (e.g., eczema or psoriasis) for 3 - 4 years prior to the diagnosis of MF<ref>{{Cite journal\|last=Kim\|first=Youn H.\|last2=Liu\|first2=Howard L.\|last3=Mraz-Gernhard\|first3=Serena\|last4=Varghese\|first4=Anna\|last5=Hoppe\|first5=Richard T.\|date=2003-07-01\|title=Long-term Outcome of 525 Patients With Mycosis Fungoides and Sézary Syndrome: Clinical Prognostic Factors and Risk for Disease Progression\|url=http://archderm.jamanetwork.com/article.aspx?doi=10.1001/archderm.139.7.857\|journal=Archives of Dermatology\|language=en\|volume=139\|issue=7\|doi=10.1001/archderm.139.7.857\|issn=0003-987X}}</ref>		*May have symptoms and/or diagnosis of other inflammatory skin diseases (e.g., eczema or psoriasis) for 3 - 4 years prior to the diagnosis of MF<ref>{{Cite journal\|last=Kim\|first=Youn H.\|last2=Liu\|first2=Howard L.\|last3=Mraz-Gernhard\|first3=Serena\|last4=Varghese\|first4=Anna\|last5=Hoppe\|first5=Richard T.\|date=2003-07-01\|title=Long-term Outcome of 525 Patients With Mycosis Fungoides and Sézary Syndrome: Clinical Prognostic Factors and Risk for Disease Progression\|url=http://archderm.jamanetwork.com/article.aspx?doi=10.1001/archderm.139.7.857\|journal=Archives of Dermatology\|language=en\|volume=139\|issue=7\|doi=10.1001/archderm.139.7.857\|issn=0003-987X}}</ref>

			<blockquote class="blockedit">
			<center><span style="color:Maroon">'''End of V4 Section'''</span>
			----
	</blockquote>		</blockquote>
	==Sites of Involvement==		==Sites of Involvement==
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	<blockquote class='blockedit'>{{Box-round\|title=v4:Chromosomal Rearrangements (Gene Fusions)\|The content below was from the old template. Please incorporate above.}}		<blockquote class='blockedit'>{{Box-round\|title=v4:Chromosomal Rearrangements (Gene Fusions)\|The content below was from the old template. Please incorporate above.}}</blockquote>

	*No consistent gene fusions		*No consistent gene fusions
	*''CD28-CTLA4'' gene fusion identified, resulting in activation of TCR signaling <ref>{{Cite journal\|last=Ungewickell\|first=Alexander\|last2=Bhaduri\|first2=Aparna\|last3=Rios\|first3=Eon\|last4=Reuter\|first4=Jason\|last5=Lee\|first5=Carolyn S\|last6=Mah\|first6=Angela\|last7=Zehnder\|first7=Ashley\|last8=Ohgami\|first8=Robert\|last9=Kulkarni\|first9=Shashikant\|date=2015-09\|title=Genomic analysis of mycosis fungoides and Sézary syndrome identifies recurrent alterations in TNFR2\|url=http://www.nature.com/articles/ng.3370\|journal=Nature Genetics\|language=en\|volume=47\|issue=9\|pages=1056–1060\|doi=10.1038/ng.3370\|issn=1061-4036\|pmc=PMC6091217\|pmid=26258847}}</ref>		*''CD28-CTLA4'' gene fusion identified, resulting in activation of TCR signaling <ref>{{Cite journal\|last=Ungewickell\|first=Alexander\|last2=Bhaduri\|first2=Aparna\|last3=Rios\|first3=Eon\|last4=Reuter\|first4=Jason\|last5=Lee\|first5=Carolyn S\|last6=Mah\|first6=Angela\|last7=Zehnder\|first7=Ashley\|last8=Ohgami\|first8=Robert\|last9=Kulkarni\|first9=Shashikant\|date=2015-09\|title=Genomic analysis of mycosis fungoides and Sézary syndrome identifies recurrent alterations in TNFR2\|url=http://www.nature.com/articles/ng.3370\|journal=Nature Genetics\|language=en\|volume=47\|issue=9\|pages=1056–1060\|doi=10.1038/ng.3370\|issn=1061-4036\|pmc=PMC6091217\|pmid=26258847}}</ref>

			<blockquote class="blockedit">
			<center><span style="color:Maroon">'''End of V4 Section'''</span>
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	* Individual Region Genomic Gain/Loss/LOH		* Individual Region Genomic Gain/Loss/LOH
	* Characteristic Chromosomal Patterns		* Characteristic Chromosomal Patterns
	* Gene Mutations (SNV/INDEL)}}		* Gene Mutations (SNV/INDEL)}}</blockquote>
	The staging system for mycosis fungoides, which also includes Sézary syndrome, was updated in 2007 by the International Society for Cutaneous Lymphomas (ISCL) and the European Organization of Research and Treatment of Cancer (EORTC) <ref>{{Cite journal\|last=Olsen\|first=Elise\|last2=Vonderheid\|first2=Eric\|last3=Pimpinelli\|first3=Nicola\|last4=Willemze\|first4=Rein\|last5=Kim\|first5=Youn\|last6=Knobler\|first6=Robert\|last7=Zackheim\|first7=Herschel\|last8=Duvic\|first8=Madeleine\|last9=Estrach\|first9=Teresa\|date=2007-09-15\|title=Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)\|url=https://pubmed.ncbi.nlm.nih.gov/17540844\|journal=Blood\|volume=110\|issue=6\|pages=1713–1722\|doi=10.1182/blood-2007-03-055749\|issn=0006-4971\|pmid=17540844}}</ref>. The revised TNMB staging of MF/SS determines management and treatment, and has been demonstrated to have prognostic significance. <ref name=":0" /> The TNMB staging forms the basis for a risk-adapted approach to treatment of mycosis fungoides.		The staging system for mycosis fungoides, which also includes Sézary syndrome, was updated in 2007 by the International Society for Cutaneous Lymphomas (ISCL) and the European Organization of Research and Treatment of Cancer (EORTC) <ref>{{Cite journal\|last=Olsen\|first=Elise\|last2=Vonderheid\|first2=Eric\|last3=Pimpinelli\|first3=Nicola\|last4=Willemze\|first4=Rein\|last5=Kim\|first5=Youn\|last6=Knobler\|first6=Robert\|last7=Zackheim\|first7=Herschel\|last8=Duvic\|first8=Madeleine\|last9=Estrach\|first9=Teresa\|date=2007-09-15\|title=Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)\|url=https://pubmed.ncbi.nlm.nih.gov/17540844\|journal=Blood\|volume=110\|issue=6\|pages=1713–1722\|doi=10.1182/blood-2007-03-055749\|issn=0006-4971\|pmid=17540844}}</ref>. The revised TNMB staging of MF/SS determines management and treatment, and has been demonstrated to have prognostic significance. <ref name=":0" /> The TNMB staging forms the basis for a risk-adapted approach to treatment of mycosis fungoides.
	{\| class="wikitable"		{\| class="wikitable"
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			<blockquote class="blockedit">
			<center><span style="color:Maroon">'''End of V4 Section'''</span>
			----
	</blockquote>		</blockquote>
	==Individual Region Genomic Gain / Loss / LOH==		==Individual Region Genomic Gain / Loss / LOH==
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	Mycosis fungoides does not have a defining disease specific molecular abnormality. However, the molecular profile of mycosis fungoides and other CTCLs continues to be investigated.		Mycosis fungoides does not have a defining disease specific molecular abnormality. However, the molecular profile of mycosis fungoides and other CTCLs continues to be investigated.
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	<blockquote class='blockedit'>{{Box-round\|title=Unassigned References\|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}<ref>{{Cite journal\|last=Mao\|first=X.\|last2=Lillington\|first2=D.\|last3=Scarisbrick\|first3=J. J.\|last4=Mitchell\|first4=T.\|last5=Czepulkowski\|first5=B.\|last6=Russell-Jones\|first6=R.\|last7=Young\|first7=B.\|last8=Whittaker\|first8=S. J.\|date=2002-09\|title=Molecular cytogenetic analysis of cutaneous T-cell lymphomas: identification of common genetic alterations in Sézary syndrome and mycosis fungoides\|url=https://pubmed.ncbi.nlm.nih.gov/12207585\|journal=The British Journal of Dermatology\|volume=147\|issue=3\|pages=464–475\|doi=10.1046/j.1365-2133.2002.04966.x\|issn=0007-0963\|pmid=12207585}}</ref></blockquote>		<blockquote class='blockedit'>{{Box-round\|title=Unassigned References\|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref>{{Cite journal\|last=Mao\|first=X.\|last2=Lillington\|first2=D.\|last3=Scarisbrick\|first3=J. J.\|last4=Mitchell\|first4=T.\|last5=Czepulkowski\|first5=B.\|last6=Russell-Jones\|first6=R.\|last7=Young\|first7=B.\|last8=Whittaker\|first8=S. J.\|date=2002-09\|title=Molecular cytogenetic analysis of cutaneous T-cell lymphomas: identification of common genetic alterations in Sézary syndrome and mycosis fungoides\|url=https://pubmed.ncbi.nlm.nih.gov/12207585\|journal=The British Journal of Dermatology\|volume=147\|issue=3\|pages=464–475\|doi=10.1046/j.1365-2133.2002.04966.x\|issn=0007-0963\|pmid=12207585}}</ref><blockquote class="blockedit">
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	==Characteristic Chromosomal Patterns==		==Characteristic Chromosomal Patterns==
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	Complex chromosomal abnormalities have been identified in mycosis fungoides, usually in tumor stage. Specific translocations have not been identified.		Complex chromosomal abnormalities have been identified in mycosis fungoides, usually in tumor stage. Specific translocations have not been identified.

			<blockquote class="blockedit">
			<center><span style="color:Maroon">'''End of V4 Section'''</span>
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	==Gene Mutations (SNV / INDEL)==		==Gene Mutations (SNV / INDEL)==
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	Although no disease specific molecular abnormalities exist in mycosis fungoides, but some changes are seen more frequently than others.<ref name=":5">{{Cite journal\|last=Walia\|first=Ritika\|last2=Yeung\|first2=Cecilia C. S.\|date=2020-01-22\|title=An Update on Molecular Biology of Cutaneous T Cell Lymphoma\|url=https://www.frontiersin.org/article/10.3389/fonc.2019.01558/full\|journal=Frontiers in Oncology\|volume=9\|pages=1558\|doi=10.3389/fonc.2019.01558\|issn=2234-943X\|pmc=PMC6987372\|pmid=32039026}}</ref> Single copy number variations (SCNV) are important mutational drivers for CTCLs and mycosis fungoides and are seen with greater frequency than somatic single nucleotide variations (SSNV) when compared to other cancers with significantly higher SCNV/SSNV ratios.<ref name=":6" /> <ref name=":3" /> Alterations in tumor suppressor genes are frequently implication the pathogenesis of mycosis fungoides, and more than 90% of variants arise from copy number alterations. <ref name=":6" />		Although no disease specific molecular abnormalities exist in mycosis fungoides, but some changes are seen more frequently than others.<ref name=":5">{{Cite journal\|last=Walia\|first=Ritika\|last2=Yeung\|first2=Cecilia C. S.\|date=2020-01-22\|title=An Update on Molecular Biology of Cutaneous T Cell Lymphoma\|url=https://www.frontiersin.org/article/10.3389/fonc.2019.01558/full\|journal=Frontiers in Oncology\|volume=9\|pages=1558\|doi=10.3389/fonc.2019.01558\|issn=2234-943X\|pmc=PMC6987372\|pmid=32039026}}</ref> Single copy number variations (SCNV) are important mutational drivers for CTCLs and mycosis fungoides and are seen with greater frequency than somatic single nucleotide variations (SSNV) when compared to other cancers with significantly higher SCNV/SSNV ratios.<ref name=":6" /> <ref name=":3" /> Alterations in tumor suppressor genes are frequently implication the pathogenesis of mycosis fungoides, and more than 90% of variants arise from copy number alterations. <ref name=":6" />
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			<blockquote class="blockedit">
			<center><span style="color:Maroon">'''End of V4 Section'''</span>
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	==Epigenomic Alterations==		==Epigenomic Alterations==
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			<center><span style="color:Maroon">'''End of V4 Section'''</span>
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	==Genetic Diagnostic Testing Methods==		==Genetic Diagnostic Testing Methods==

Bailey.Glen at 22:36, 6 September 2024

2024-09-06T22:36:42Z

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	__TOC__		__TOC__

	==~~Cancer Category / Type~~==		==WHO Classification of Disease==

	*[[HAEM4:Mature T- and NK-cell ~~Neoplasms]]~~		{\| class="wikitable"
			!Structure
	~~==Cancer Sub~~-~~Classification / Subtype==~~		!Disease
			\|-
	*Mycosis Fungoides (MF)		\|Book
			\|Haematolymphoid Tumours (5th ed.)
			\|-
			\|Category
			\|T-cell and NK-cell lymphoid proliferations and lymphomas
			\|-
			\|Family
			\|Mature T-cell and NK-cell neoplasms
			\|-
			\|Type
			\|Primary cutaneous T-cell lymphoid proliferations and lymphomas
			\|-
			\|Subtype(s)
			\|Mycosis fungoides
			\|}

	==Definition / Description of Disease==		==Definition / Description of Disease==

Bailey.Glen at 22:03, 6 September 2024

2024-09-06T22:03:56Z

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Bailey.Glen at 19:32, 13 December 2023

2023-12-13T19:32:14Z

← Older revision		Revision as of 14:32, 13 December 2023
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	<blockquote class='blockedit'>{{Box-round\|title=HAEM5 Conversion Notes\|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Mycosis Fungoides]].		<blockquote class='blockedit'>{{Box-round\|title=HAEM5 Conversion Notes\|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Mycosis Fungoides]].
	}}</blockquote>		}}</blockquote>

			<span style="color:#0070C0">(General Instructions – The main focus of these pages is the clinically significant genetic alterations in each disease type. Use [https://www.genenames.org/ <u>HUGO-approved gene names and symbols</u>] (italicized when appropriate), [https://varnomen.hgvs.org/ HGVS-based nomenclature for variants], as well as generic names of drugs and testing platforms or assays if applicable. Please complete tables whenever possible and do not delete them (add N/A if not applicable in the table and delete the examples). Please do not delete or alter the section headings. The use of bullet points alongside short blocks of text rather than only large paragraphs is encouraged. Additional instructions below in italicized blue text should not be included in the final page content. Please also see </span><u>[[Author_Instructions]]</u><span style="color:#0070C0"> and [[Frequently Asked Questions (FAQs)\|<u>FAQs</u>]] as well as contact your [[Leadership\|<u>Associate Editor</u>]] or [mailto:CCGA@cancergenomics.org <u>Technical Support</u>])</span>

	==Primary Author(s)*==		==Primary Author(s)*==