HAEM5:Mycosis fungoides: Difference between revisions

[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (5th ed.)]]

<blockquote class='blockedit'>{{Box-round|title=HAEM5 Conversion Notes|This page was converted to the new template on 2023-12-04. The original page can be found at [[HAEM4:Mycosis Fungoides]].

 

==Cancer Category / Type==

 

 

==Cancer Sub-Classification / Subtype==

 

*Mycosis Fungoides (MF)

 

 

MF is a primary cutaneous T-cell lymphoma (CTCL) of epidermotropic small to medium-sized T lymphocytes presenting with skin patches that progress slowly to plaques and tumors.  The disease is postulated to be caused by skin-homing mature T cells, the majority of which are CD4-positive.  Sézary syndrome (SS) is often used to refer to the leukemic phase of mycosis fungoides.  However, recently studies have suggested that there are major genomic and phenotypical differences between these two entities. <ref>{{Cite journal|last=Campbell|first=James J.|last2=Clark|first2=Rachael A.|last3=Watanabe|first3=Rei|last4=Kupper|first4=Thomas S.|date=2010-08-05|title=Sézary syndrome and mycosis fungoides arise from distinct T-cell subsets: a biologic rationale for their distinct clinical behaviors|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2918332/|journal=Blood|volume=116|issue=5|pages=767–771|doi=10.1182/blood-2009-11-251926|issn=0006-4971|pmc=2918332|pmid=20484084}}</ref> 

 

*Cutaneous T-cell lymphoma

*Alibert-Bazin syndrome

 

*Most common CTCL subtype (54% of CTCL)<ref>{{Cite journal|last=Bradford|first=Porcia T.|last2=Devesa|first2=Susan S.|last3=Anderson|first3=William F.|last4=Toro|first4=Jorge R.|date=2009-05-21|title=Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases|url=https://pubmed.ncbi.nlm.nih.gov/19279331|journal=Blood|volume=113|issue=21|pages=5064–5073|doi=10.1182/blood-2008-10-184168|issn=1528-0020|pmc=2686177|pmid=19279331}}</ref>

|'''Signs and Symptoms'''

|EXAMPLE Asymptomatic (incidental finding on complete blood counts)

 

 

 

|'''Laboratory Findings'''

|EXAMPLE Cytopenias

 

EXAMPLE Lymphocytosis (low level)

!Finding!!Marker

|Positive (T-cell lineage markers)||CD3, CD4, CD45RO

|Variable expression||CD2, CD5, CD7 (often lost, significant only if 90% loss)

|Markers with aberrant expression

|CD8 (CD4:CD8 ratio of 10:1 suggestive of mycosis fungoides), CD30 (may indicate transformation)

Put your text here and fill in the table

!Chromosomal Rearrangement!!Genes in Fusion (5’ or 3’ Segments)!!Pathogenic Derivative!!Prevalence

!Diagnostic Significance (Yes, No or Unknown)

!Prognostic Significance (Yes, No or Unknown)

|EXAMPLE t(9;22)(q34;q11.2)||EXAMPLE 3'ABL1 / 5'BCR||EXAMPLE der(22)||EXAMPLE 20% (COSMIC)

|Yes

|No

|Yes

|EXAMPLE

 

|}

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<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).|Please incorporate this section into the relevant tables found in:

* Gene Mutations (SNV/INDEL)}}

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.

==Individual Region Genomic Gain / Loss / LOH==

!Chr #!!Gain / Loss / Amp / LOH!!Minimal Region Genomic Coordinates [Genome Build]!!Minimal Region Cytoband

!Diagnostic Significance (Yes, No or Unknown)

!Prognostic Significance (Yes, No or Unknown)

!Therapeutic Significance (Yes, No or Unknown)

!Notes

|EXAMPLE

 

|EXAMPLE Loss

|EXAMPLE

 

chr7:1- 159,335,973 [hg38]

 

|Yes

|Yes

|No

|EXAMPLE

 

Presence of monosomy 7 (or 7q deletion) is sufficient for a diagnosis of AML with MDS-related changes when there is ≥20% blasts and no prior therapy (add reference).  Monosomy 7/7q deletion is associated with a poor prognosis in AML (add reference).

|EXAMPLE

 

|EXAMPLE Gain

|EXAMPLE

 

chr8:1-145,138,636 [hg38]

 

|No

|No

|No

|EXAMPLE

 

Common recurrent secondary finding for t(8;21) (add reference).

<blockquote class='blockedit'>{{Box-round|title=v4:Genomic Gain/Loss/LOH|The content below was from the old template. Please incorporate above.}}

<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>

==Characteristic Chromosomal Patterns==

!Diagnostic Significance (Yes, No or Unknown)

!Prognostic Significance (Yes, No or Unknown)

!Therapeutic Significance (Yes, No or Unknown)

!Notes

|EXAMPLE

 

|Yes

|EXAMPLE:

 

See chromosomal rearrangements table as this pattern is due to an unbalanced derivative translocation associated with oligodendroglioma (add reference).

<blockquote class='blockedit'>{{Box-round|title=v4:Characteristic Chromosomal Aberrations / Patterns|The content below was from the old template. Please incorporate above.}}

==Gene Mutations (SNV / INDEL)==

!Gene; Genetic Alteration!!'''Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other)'''!!'''Prevalence (COSMIC /  TCGA / Other)'''!!'''Concomitant Mutations'''!!'''Mutually Exclusive Mutations'''

!'''Diagnostic Significance (Yes, No or Unknown)'''

!Prognostic Significance (Yes, No or Unknown)

!Therapeutic Significance (Yes, No or Unknown)

!Notes

|EXAMPLE: TP53; Variable LOF mutations

EXAMPLE:

 

EGFR; Exon 20 mutations

 

EXAMPLE: BRAF; Activating mutations

|EXAMPLE: TSG

|EXAMPLE: 20% (COSMIC)

 

EXAMPLE: 30% (add Reference)

|EXAMPLE: IDH1 R123H

|EXAMPLE: EGFR amplification

|}

Note: A more extensive list of mutations can be found in cBioportal (https://www.cbioportal.org/), COSMIC (https://cancer.sanger.ac.uk/cosmic), ICGC (https://dcc.icgc.org/) and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.

 

<blockquote class='blockedit'>{{Box-round|title=v4:Gene Mutations (SNV/INDEL)|The content below was from the old template. Please incorporate above.}}

Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Can include references in the table.'')</span>

|EXAMPLE: BRAF and MAP2K1; Activating mutations

|EXAMPLE: MAPK signaling

|EXAMPLE: Increased cell growth and proliferation

|EXAMPLE: CDKN2A; Inactivating mutations

|EXAMPLE: Cell cycle regulation

|EXAMPLE: Unregulated cell division

|EXAMPLE:  KMT2C and ARID1A; Inactivating mutations

|EXAMPLE:  Histone modification, chromatin remodeling

|EXAMPLE:  Abnormal gene expression program

<blockquote class='blockedit'>{{Box-round|title=v4:Genes and Main Pathways Involved|The content below was from the old template. Please incorporate above.}}

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