HAEM5:Mycosis fungoides: Difference between revisions
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<blockquote class= | <blockquote class="blockedit">{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Mycosis Fungoides]]. | ||
}}</blockquote> | }}</blockquote> | ||
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Jane Scribner, MD and Daynna J. Wolff, PhD | Jane Scribner, MD and Daynna J. Wolff, PhD | ||
==WHO Classification of Disease== | ==WHO Classification of Disease== | ||
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|} | |} | ||
== | ==Related Terminology== | ||
{| class="wikitable" | {| class="wikitable" | ||
|+ | |+ | ||
|Acceptable | |Acceptable | ||
| | |N/A | ||
|- | |- | ||
|Not Recommended | |Not Recommended | ||
| | |Classic mycosis fungoides (type Alibert–Bazin); Woringer–Kolopp disease | ||
|} | |} | ||
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|} | |} | ||
<blockquote class= | <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 | ||
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<blockquote class= | <blockquote class="blockedit">{{Box-round|title=v4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).|Please incorporate this section into the relevant tables found in: | ||
* Chromosomal Rearrangements (Gene Fusions) | * Chromosomal Rearrangements (Gene Fusions) | ||
* Individual Region Genomic Gain/Loss/LOH | * Individual Region Genomic Gain/Loss/LOH | ||
* Characteristic Chromosomal Patterns | * Characteristic Chromosomal Patterns | ||
* Gene Mutations (SNV/INDEL)}}</blockquote> | * 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">{{Cite journal|last=Agar|first=Nita Sally|last2=Wedgeworth|first2=Emma|last3=Crichton|first3=Siobhan|last4=Mitchell|first4=Tracey J.|last5=Cox|first5=Michael|last6=Ferreira|first6=Silvia|last7=Robson|first7=Alistair|last8=Calonje|first8=Eduardo|last9=Stefanato|first9=Catherine M.|date=2010-11-01|title=Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: validation of the revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer staging proposal|url=https://pubmed.ncbi.nlm.nih.gov/20855822|journal=Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology|volume=28|issue=31|pages=4730–4739|doi=10.1200/JCO.2009.27.7665|issn=1527-7755|pmid=20855822}}</ref> The TNMB staging forms the basis for a risk-adapted approach to treatment of mycosis fungoides. | ||
{| class="wikitable" | {| class="wikitable" | ||
|+ | |+ | ||
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{| class="wikitable sortable" | {| class="wikitable sortable" | ||
|- | |- | ||
!Chr #!! | !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 | ||
! | !Established Clinical Significance Per Guidelines - Yes or No (Source) | ||
! | !Clinical Relevance Details/Other Notes | ||
|- | |- | ||
|<span class="blue-text">EXAMPLE:</span> | |<span class="blue-text">EXAMPLE:</span> | ||
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<blockquote class= | <blockquote class="blockedit">{{Box-round|title=v4:Genomic Gain/Loss/LOH|The content below was from the old template. Please incorporate above.}}</blockquote> | ||
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= | <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"> | ||
<center><span style="color:Maroon">'''End of V4 Section'''</span> | <center><span style="color:Maroon">'''End of V4 Section'''</span> | ||
---- | ---- | ||
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!Chromosomal Pattern | !Chromosomal Pattern | ||
!Molecular Pathogenesis | !Molecular Pathogenesis | ||
! | !Prevalence - | ||
Common >20%, Recurrent 5-20% or Rare <5% (Disease) | |||
! | !Diagnostic, Prognostic, and Therapeutic Significance - D, P, T | ||
! | !Established Clinical Significance Per Guidelines - Yes or No (Source) | ||
! | !Clinical Relevance Details/Other Notes | ||
|- | |- | ||
|<span class="blue-text">EXAMPLE:</span> | |<span class="blue-text">EXAMPLE:</span> | ||
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|} | |} | ||
<blockquote class= | <blockquote class="blockedit">{{Box-round|title=v4:Characteristic Chromosomal Aberrations / Patterns|The content below was from the old template. Please incorporate above.}}</blockquote> | ||
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. | ||
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{| class="wikitable sortable" | {| class="wikitable sortable" | ||
|- | |- | ||
!Gene!! | !Gene!!Genetic Alteration!!Tumor Suppressor Gene, Oncogene, Other!!Prevalence - | ||
Common >20%, Recurrent 5-20% or Rare <5% (Disease) | |||
! | !Diagnostic, Prognostic, and Therapeutic Significance - D, P, T | ||
! | !Established Clinical Significance Per Guidelines - Yes or No (Source) | ||
! | !Clinical Relevance Details/Other Notes | ||
|- | |- | ||
|<span class="blue-text">EXAMPLE:</span>''EGFR'' | |<span class="blue-text">EXAMPLE:</span>''EGFR'' | ||
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|}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content. | |}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content. | ||
<blockquote class= | <blockquote class="blockedit">{{Box-round|title=v4:Gene Mutations (SNV/INDEL)|The content below was from the old template. Please incorporate above.}}</blockquote> | ||
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= | <blockquote class="blockedit">{{Box-round|title=v4:Genes and Main Pathways Involved|The content below was from the old template. Please incorporate above.}}</blockquote> | ||
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(use the "Cite" icon at the top of the page) <span style="color:#0070C0">(''Instructions: Add each reference into the text above by clicking where you want to insert the reference, selecting the “Cite” icon at the top of the wiki page, and using the “Automatic” tab option to search by PMID to select the reference to insert. If a PMID is not available, such as for a book, please use the “Cite” icon, select “Manual” and then “Basic Form”, and include the entire reference. To insert the same reference again later in the page, select the “Cite” icon and “Re-use” to find the reference; DO NOT insert the same reference twice using the “Automatic” tab as it will be treated as two separate references. The reference list in this section will be automatically generated and sorted''</span><span style="color:#0070C0">''.''</span><span style="color:#0070C0">)</span> <references /> | (use the "Cite" icon at the top of the page) <span style="color:#0070C0">(''Instructions: Add each reference into the text above by clicking where you want to insert the reference, selecting the “Cite” icon at the top of the wiki page, and using the “Automatic” tab option to search by PMID to select the reference to insert. If a PMID is not available, such as for a book, please use the “Cite” icon, select “Manual” and then “Basic Form”, and include the entire reference. To insert the same reference again later in the page, select the “Cite” icon and “Re-use” to find the reference; DO NOT insert the same reference twice using the “Automatic” tab as it will be treated as two separate references. The reference list in this section will be automatically generated and sorted''</span><span style="color:#0070C0">''.''</span><span style="color:#0070C0">)</span> <references /> | ||
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==Notes== | ==Notes== | ||
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<nowiki>*</nowiki>''Citation of this Page'': “Mycosis fungoides”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Mycosis_fungoides</nowiki>. | <nowiki>*</nowiki>''Citation of this Page'': “Mycosis fungoides”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Mycosis_fungoides</nowiki>. | ||
[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases M]] | [[Category:HAEM5]] | ||
[[Category:DISEASE]] | |||
[[Category:Diseases M]] | |||