HAEM5:Primary cutaneous follicle centre lymphoma: Difference between revisions

{{DISPLAYTITLE:Primary cutaneous follicle centre lymphoma}}

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

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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>

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The t(14;18)(q32;q21), ''IGH''/''BCL2'' translocation, the genetic hallmark of nodal follicular lymphoma, is rare in primary cutaneous follicle center lymphoma<ref name=":0">{{Cite journal|last=Gángó|first=Ambrus|last2=Bátai|first2=Bence|last3=Varga|first3=Martin|last4=Kapczár|first4=Dóra|last5=Papp|first5=Gergő|last6=Marschalkó|first6=Márta|last7=Kuroli|first7=Enikő|last8=Schneider|first8=Tamás|last9=Csomor|first9=Judit|date=2018-10|title=Concomitant 1p36 deletion and TNFRSF14 mutations in primary cutaneous follicle center lymphoma frequently expressing high levels of EZH2 protein|url=https://pubmed.ncbi.nlm.nih.gov/29858685|journal=Virchows Archiv: An International Journal of Pathology|volume=473|issue=4|pages=453–462|doi=10.1007/s00428-018-2384-3|issn=1432-2307|pmid=29858685}}</ref><ref>{{Cite journal|last=Goodlad|first=John R.|last2=Krajewski|first2=Andrew S.|last3=Batstone|first3=Paul J.|last4=McKay|first4=Pam|last5=White|first5=Jo M.|last6=Benton|first6=E. Claire|last7=Kavanagh|first7=Gina M.|last8=Lucraft|first8=Helen H.|last9=Scotland and Newcastle Lymphoma Group|date=2003-12|title=Primary cutaneous diffuse large B-cell lymphoma: prognostic significance of clinicopathological subtypes|url=https://pubmed.ncbi.nlm.nih.gov/14657713|journal=The American Journal of Surgical Pathology|volume=27|issue=12|pages=1538–1545|doi=10.1097/00000478-200312000-00006|issn=0147-5185|pmid=14657713}}</ref>.

* Chromosomal Rearrangements (Gene Fusions)

* Individual Region Genomic Gain/Loss/LOH

* Gene Mutations (SNV/INDEL)}}</blockquote>

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In a study of the genetic abnormalities of primary cutaneous follicle center lymphoma, 1p36 deletion was reported to occur in 22% (5/21) and ''BCL2'' gene break in 10% (2/20) of the cases. ''TNFRSF14'' nonsense and missense mutations were detected in 4/17 (23.5%) cases with concomitant 1p36 deletion in 2 cases.  In 43% (9/21) of the cases, high EZH2 protein expression with a ''BCL2'' negative phenotype was detected<ref name=":0" />. In another study that investigated  57 patients with PCFCL, 1 case was found to have ''BCL2'' chromosomal amplification, 4 cases had ''IGH''/''BCL2 translocation'', and 1 case had ''IGH/MALT1'' translocation<ref>{{Cite journal|last=Abdul-Wahab|first=Alya|last2=Tang|first2=Soo-Yong|last3=Robson|first3=Alistair|last4=Morris|first4=Stephen|last5=Agar|first5=Nita|last6=Wain|first6=E. Mary|last7=Child|first7=Fiona|last8=Scarisbrick|first8=Julia|last9=Neat|first9=Michael|date=2014-06|title=Chromosomal anomalies in primary cutaneous follicle center cell lymphoma do not portend a poor prognosis|url=https://doi.org/10.1016/j.jaad.2014.01.862|journal=Journal of the American Academy of Dermatology|volume=70|issue=6|pages=1010–1020|doi=10.1016/j.jaad.2014.01.862|issn=0190-9622}}</ref>. In a case report of an aggressive PCFCL, ''c-MYC'' translocation and ''CDKN2A'' (9p21) deletion were detected<ref>{{Cite journal|last=Tsang|first=Hamilton C.|last2=Mathew|first2=Susan|last3=Magro|first3=Cynthia M.|date=2017-03|title=An Aggressive Primary Cutaneous Follicle Center Lymphoma With c-MYC Translocation and CDKN2A (9p21) Deletion: A Case Report and Review of the Literature|url=https://pubmed.ncbi.nlm.nih.gov/27759694|journal=The American Journal of Dermatopathology|volume=39|issue=3|pages=e44–e49|doi=10.1097/DAD.0000000000000738|issn=1533-0311|pmid=27759694}}</ref>.   

|}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.

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''BCL2-''mediated apoptosis pathway and NF-κB pathway   

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==Notes==

          

<nowiki>*</nowiki>''Citation of this Page'': “Primary cutaneous follicle centre lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Primary_cutaneous_follicle_centre_lymphoma</nowiki>.