HAEM5:Primary cutaneous follicle centre lymphoma: Difference between revisions

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Linlin Gao, MD, PhD and Shivani Golem, PhD, FACMG

==WHO Classification of Disease==

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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>.   

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|<span class="blue-text">EXAMPLE:</span>''EGFR''

|}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   

(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 />

==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>.