Bailey.Glen at 21:36, 4 December 2023

2023-12-04T21:36:23Z

← Older revision		Revision as of 16:36, 4 December 2023
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	==Cancer Category/Type==		==Cancer Category/Type==

	*[[Mature B-Cell Neoplasms]]		*[[HAEM4:Mature B-Cell Neoplasms]]

	==Cancer Sub-Classification / Subtype==		==Cancer Sub-Classification / Subtype==

Bailey.Glen at 18:53, 3 November 2023

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			{{DISPLAYTITLE:Primary Cutaneous Follicle Centre Lymphoma}}


			<blockquote class='blockedit'>{{Box-round\|title=PREVIOUS EDITION\|This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition [[HAEM5:Table_of_Contents\|Table of Contents]].
			}}</blockquote>
	==Primary Author(s)*==		==Primary Author(s)*==

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	<references />		<references />
			[[Category:HAEM4]] [[Category:DISEASE]]

Bailey.Glen: Created page with "==Primary Author(s)== Linlin Gao, MD, PhD and Shivani Golem, PhD, FACMG TOC ==Cancer Category/Type== Mature B-Cell Neoplasms ==Cancer Sub-Classification / Subty..."

2023-11-03T18:15:30Z

Created page with "==Primary Author(s)*== Linlin Gao, MD, PhD and Shivani Golem, PhD, FACMG __TOC__ ==Cancer Category/Type== *Mature B-Cell Neoplasms ==Cancer Sub-Classification / Subty..."

New page

==Primary Author(s)*==

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

__TOC__

==Cancer Category/Type==

*[[Mature B-Cell Neoplasms]]

==Cancer Sub-Classification / Subtype==

*Primary Cutaneous Follicle Center Lymphoma

==Definition / Description of Disease==

Primary cutaneous follicle center lymphoma (PCFCL) is a tumor arising in skin composed of germinal center B cells, including centrocytes and centroblasts<ref name=":1">{{Cite journal|last=Gulia|first=Andrea|last2=Saggini|first2=Andrea|last3=Wiesner|first3=Thomas|last4=Fink-Puches|first4=Regina|last5=Argenyi|first5=Zsolt|last6=Ferrara|first6=Gerardo|last7=Müller|first7=Cornelia S.L.|last8=Vale|first8=Esmeralda|last9=Cerroni|first9=Lorenzo|date=2011-11|title=Clinicopathologic features of early lesions of primary cutaneous follicle center lymphoma, diffuse type: Implications for early diagnosis and treatment|url=https://doi.org/10.1016/j.jaad.2010.06.059|journal=Journal of the American Academy of Dermatology|volume=65|issue=5|pages=991–1000.e7|doi=10.1016/j.jaad.2010.06.059|issn=0190-9622}}</ref>.

==Synonyms / Terminology==

*Reticulohistiocytoma of the dorsum
*Crosti lymphoma

==Epidemiology / Prevalence==

*It accounts for about 50% of primary cutaneous B-cell lymphomas with an incidence of 0.1-0.2 per 100,000 persons per year
*It mainly occurs in middle-aged adults
*Male: female ratio is approximately 1.5:1<ref name=":2">World health organization classification of tumours of haematopoietic and lymphoid tissues, revised 4th edition, Swerdlow SH, Campo E, Harris NL, et al. (Eds), IARC, Lyon 2017.</ref>

==Clinical Features==

*Usually solitary, firm, and erythematous to violaceous plaques, nodules or tumors of variable size
*Multifocal in 15% of patients
*Lesions on the trunk may be surrounded by erythematous papules
*The skin surface is usually smooth and rarely ulcerated<ref name=":2" />

==Sites of Involvement==

*Head
*Trunk

==Morphologic Features==

*Perivascular, periadnexal, or diffuse infiltrates with sparing of the epidermis
*The growth patterns include follicular, follicular and diffuse, and diffuse patterns<ref>{{Cite journal|last=Senff|first=Nancy J.|last2=Hoefnagel|first2=Juliette J.|last3=Jansen|first3=Patty M.|last4=Vermeer|first4=Maarten H.|last5=van Baarlen|first5=Joop|last6=Blokx|first6=Willeke A.|last7=Canninga-van Dijk|first7=Marijke R.|last8=Geerts|first8=Marie-Louise|last9=Hebeda|first9=Konnie M.|date=2007-04-20|title=Reclassification of 300 primary cutaneous B-Cell lymphomas according to the new WHO-EORTC classification for cutaneous lymphomas: comparison with previous classifications and identification of prognostic markers|url=https://pubmed.ncbi.nlm.nih.gov/17353548|journal=Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology|volume=25|issue=12|pages=1581–1587|doi=10.1200/JCO.2006.09.6396|issn=1527-7755|pmid=17353548}}</ref>
*The tumor is composed of centrocytes and variable numbers of centroblasts<ref name=":1" />
*In a tumor with follicular growth pattern, follicles are poorly defined and composed of monotonous follicle center cells with no polarization
**A follicular dendritic cell meshwork is present
**Tingible body macrophages are usually absent
**Mantle zones are attenuated or absent
**Proliferation rate is low
*In a tumor with diffuse growth pattern, tumor cells are mainly large centrocytes, some of which are multilobated or spindle-shaped<ref name=":2" />
**Variable numbers of large centroblasts
**Follicular dendritic cell meshwork may be lost
**Proliferation rate is generally high

==Immunophenotype==

{| class="wikitable sortable"
|-
!Finding
!Marker
|-
|Positive (universal)
|CD20, CD79a, BCL6
|-
|Positive (tumor with a follicular growth pattern)
|CD10
|-
|Negative (universal)
|CD5, CD43
|-
|Negative (most cases)
|BCL2, MUM1, FOXP1
|}

==Chromosomal Rearrangements (Gene Fusions)==

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>.
{| class="wikitable sortable"
!Chromosomal Rearrangement
!Genes in Fusion (5’ or 3’ Segments)
!Pathogenic Derivative
!Prevalence
|-
|t(14;18)(q32;q21)
|5'BCL2/3'IGH
|der(14)
|7%
|-
|t(14;18)(q32;q21)
|5'MALT1/3'IGH
|der(14)
|2%
|}

==Characteristic Chromosomal Aberrations / Patterns==

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>.

==Genomic Gain/Loss/LOH==

{| class="wikitable sortable"
|-
!Chromosome Number!!Gain/Loss/Amp/LOH!!Region
|-
|1||Loss||chr 1p36
|-
|18
|Amplification
|chr 18q21.33
|}

==Gene Mutations (SNV/INDEL)==

{| class="wikitable sortable"
|-
!Gene!!Mutation!!Oncogene/Tumor Suppressor/Other!!Presumed Mechanism (LOF/GOF/Other; Driver/Passenger)!!Prevalence (COSMIC/TCGA/Other)
|-
|''TNFRSF14''||W12Ter||Tumor suppressor||LOF||17.6%
|-
|''TNFRSF14''
|C53G
|Tumor suppressor
|LOF
|6%
|}

==Epigenomics (Methylation)==

*Not known in this specific subgroup.

==Genes and Main Pathways Involved==

''BCL2-''mediated apoptosis pathway and NF-κB pathway
==Diagnostic Testing Methods==

*Fluorescence in situ hybridization with ''BCL2'' break apart and 1p36/1q25 dual color probes
*Polymerase chain reaction for ''IGH'' rearrangements
*Chromosomal microarray or karyotype analysis for 1p36 deletion
*DNA sequencing for ''TNFRSF14'' mutations

==Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications)==

*Chromosomal abnormalities in PCFCL involving ''BCL2'' or ''MALT1'' do not correlate with a poor prognosis<ref name=":2" />.

==Familial Forms==

*Not known in this specific subgroup.

==Other Information==

Prognosis: Excellent prognosis with a 5-year survival rate >90%<ref>{{Cite journal|last=Lucioni|first=Marco|last2=Berti|first2=Emilio|last3=Arcaini|first3=Luca|last4=Croci|first4=Giorgio A.|last5=Maffi|first5=Aldo|last6=Klersy|first6=Catherine|last7=Goteri|first7=Gaia|last8=Tomasini|first8=Carlo|last9=Quaglino|first9=Pietro|date=2016-10|title=Primary cutaneous B-cell lymphoma other than marginal zone: clinicopathologic analysis of 161 cases: Comparison with current classification and definition of prognostic markers|url=https://pubmed.ncbi.nlm.nih.gov/27665744|journal=Cancer Medicine|volume=5|issue=10|pages=2740–2755|doi=10.1002/cam4.865|issn=2045-7634|pmc=5083727|pmid=27665744}}</ref>.

==Links==

*None

==References==

<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome.

<references />

HAEM4:Primary Cutaneous Follicle Centre Lymphoma - Revision history

Bailey.Glen at 21:36, 4 December 2023

Bailey.Glen at 18:53, 3 November 2023

Bailey.Glen: Created page with "==Primary Author(s)*== Linlin Gao, MD, PhD and Shivani Golem, PhD, FACMG __TOC__ ==Cancer Category/Type== *Mature B-Cell Neoplasms ==Cancer Sub-Classification / Subty..."

Bailey.Glen: Created page with "==Primary Author(s)== Linlin Gao, MD, PhD and Shivani Golem, PhD, FACMG TOC ==Cancer Category/Type== Mature B-Cell Neoplasms ==Cancer Sub-Classification / Subty..."