Compendium of Cancer Genome Aberrations

HAEM5:Paediatric nodal marginal zone lymphoma: Difference between revisions

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{{DISPLAYTITLE:Paediatric nodal marginal zone lymphoma}}
{{DISPLAYTITLE:Paediatric nodal marginal zone lymphoma}}
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]


{{Under Construction}}
{{Under Construction}}


<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:Paediatric Nodal Marginal Zone Lymphoma]].
<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:Paediatric Nodal Marginal Zone Lymphoma]].
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==Primary Author(s)*==
==Primary Author(s)*==


* Kathleen M. Schieffer, PhD
*Kathleen M. Schieffer, PhD
* Ruthann Pfau, PhD
*Ruthann Pfau, PhD


__TOC__
__TOC__
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|Subtype(s)
|Subtype(s)
|Paediatric nodal marginal zone lymphoma
|Paediatric nodal marginal zone lymphoma
|}
==Definition / Description of Disease==
* Paediatric nodal marginal zone lymphoma (pNMZL) is a rare and distinct entity of [[HAEM5:Nodal marginal zone lymphoma|NMZL]] seen in the pediatrics and young adult population<ref name=":1">Swerdlow SH, Campo E, Harris NL et al (eds) WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. IARC Press, Lyon, pp 264-265</ref>
* pNMZL typically presents with an indolent course and localized disease<ref name=":1" />, contrary from classic NMZL seen in adults<ref name=":2">Swerdlow SH, Campo E, Harris NL et al (eds) WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. IARC Press, Lyon, pp 263-264</ref>
* Typically presents as asymptomatic, localized lymphadenopathy (Stage I)<ref name=":3">{{Cite journal|last=Koo|first=Matthew|last2=Ohgami|first2=Robert S.|date=2017-05|title=Pediatric-type Follicular Lymphoma and Pediatric Nodal Marginal Zone Lymphoma: Recent Clinical, Morphologic, Immunophenotypic, and Genetic Insights|url=https://pubmed.ncbi.nlm.nih.gov/28277421|journal=Advances in Anatomic Pathology|volume=24|issue=3|pages=128–135|doi=10.1097/PAP.0000000000000144|issn=1533-4031|pmid=28277421}}</ref><ref name=":4">{{Cite journal|last=Ronceray|first=Leila|last2=Abla|first2=Oussama|last3=Barzilai-Birenboim|first3=Shlomit|last4=Bomken|first4=Simon|last5=Chiang|first5=Alan Ks|last6=Jazbec|first6=Janez|last7=Kabickova|first7=Edita|last8=Lazic|first8=Jelena|last9=Beishuizen|first9=Auke|date=04 2018|title=Children and adolescents with marginal zone lymphoma have an excellent prognosis with limited chemotherapy or a watch-and-wait strategy after complete resection|url=https://pubmed.ncbi.nlm.nih.gov/29286565|journal=Pediatric Blood & Cancer|volume=65|issue=4|doi=10.1002/pbc.26932|issn=1545-5017|pmid=29286565}}</ref><ref name=":5">{{Cite journal|last=Makarova|first=Olga|last2=Oschlies|first2=Ilske|last3=Müller|first3=Stephanie|last4=Ruf|first4=Stephanie|last5=Zimmermann|first5=Martin|last6=Niggli|first6=Felix|last7=Attarbaschi|first7=Andishe|last8=Kabickova|first8=Edita|last9=Klapper|first9=Wolfram|date=09 2018|title=Excellent outcome with limited treatment in paediatric patients with marginal zone lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/28771659|journal=British Journal of Haematology|volume=182|issue=5|pages=735–739|doi=10.1111/bjh.14868|issn=1365-2141|pmid=28771659}}</ref>
* Laboratory testing: normal serum lactate dehydrogenase (LDH) levels<ref name=":3" /><ref name=":4" /><ref name=":5" />
* Prognosis is typically excellent<ref name=":4" /><ref name=":5" /><ref name=":6">{{Cite journal|last=Quintanilla-Martinez|first=Leticia|last2=Sander|first2=Birgitta|last3=Chan|first3=John K. C.|last4=Xerri|first4=Luc|last5=Ott|first5=German|last6=Campo|first6=Elias|last7=Swerdlow|first7=Steven H.|date=2016-02|title=Indolent lymphomas in the pediatric population: follicular lymphoma, IRF4/MUM1+ lymphoma, nodal marginal zone lymphoma and chronic lymphocytic leukemia|url=https://pubmed.ncbi.nlm.nih.gov/26416032|journal=Virchows Archiv: An International Journal of Pathology|volume=468|issue=2|pages=141–157|doi=10.1007/s00428-015-1855-z|issn=1432-2307|pmid=26416032}}</ref>
** Five year event free survival: 94±6%<ref name=":4" />
** Five year overall survival: 100%<ref name=":4" />
* Complete remission follows surgical resection in most patients with limited/localized disease<ref name=":4" /><ref name=":8" />
* Chemotherapy and radiation therapy have also been used for management of limited stage disease<ref name=":4" /><ref name=":8">{{Cite journal|displayauthors=1|last=Taddessee-Heath|first=Lekidelu|date=|title=Marginal zone B-cell lymphoma in children and young adults|url=|journal=Am J Surg Pathol|volume=24|pages=522-531|doi=10.1097/00000478-200304000-00014|pmc=PMC6324530|pmid=12657939|via=}}</ref>
==Synonyms / Terminology==
* Monocytoid B-cell lymphoma
* Parafollicular B-cell lymphoma (obsolete)
==Epidemiology / Prevalence==
* Male: Female 4-20:1<ref name=":3" /><ref name=":5" /><ref name=":6" /><ref name=":8" /><ref name=":0">{{Cite journal|last=Ozawa|first=Michael G.|last2=Bhaduri|first2=Aparna|last3=Chisholm|first3=Karen M.|last4=Baker|first4=Steven A.|last5=Ma|first5=Lisa|last6=Zehnder|first6=James L.|last7=Luna-Fineman|first7=Sandra|last8=Link|first8=Michael P.|last9=Merker|first9=Jason D.|date=10 2016|title=A study of the mutational landscape of pediatric-type follicular lymphoma and pediatric nodal marginal zone lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/27338637|journal=Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc|volume=29|issue=10|pages=1212–1220|doi=10.1038/modpathol.2016.102|issn=1530-0285|pmc=5047957|pmid=27338637}}</ref><ref name=":7">{{Cite journal|displayauthors=1|last=Liu|first=Qingyan|date=|title=Follicular lymphomas in children and young adults: a comparison of the pediatric variant with usual follicular lymphoma|url=|journal=Am J Surg Pathol|volume=37|pages=333-343|doi=10.1097/PAS.0b013e31826b9b57|pmid=23108024|via=}}</ref><ref name=":9">{{Cite journal|displayauthors=1|last=Rizzo|first=Kathryn|date=|title=Marginal zone lymphomas in children and the young adult population; characterization of genetic aberrations by FISH and RT-PCR|url=|journal=Mol Pathol|volume=23|pages=866-873|doi=10.1038/modpathol.2010.63|pmc=PMC6329460|pmid=20305621|via=}}</ref><ref name=":11">{{Cite journal|last=Ganapathi|first=Karthik A.|last2=Pittaluga|first2=Stefania|last3=Odejide|first3=Oreofe O.|last4=Freedman|first4=Arnold S.|last5=Jaffe|first5=Elaine S.|date=2014-09|title=Early lymphoid lesions: conceptual, diagnostic and clinical challenges|url=https://pubmed.ncbi.nlm.nih.gov/25176983|journal=Haematologica|volume=99|issue=9|pages=1421–1432|doi=10.3324/haematol.2014.107938|issn=1592-8721|pmc=4562530|pmid=25176983}}</ref>
* Median age ~16-17 years (range 1.5-44 years)<ref name=":3" /><ref name=":5" /><ref name=":6" /><ref name=":8" /><ref name=":0" /><ref name=":7" /><ref name=":9" /><ref name=":11" /><ref>{{Cite journal|last=Gitelson|first=Elena|last2=Al-Saleem|first2=Tahseen|last3=Robu|first3=Valentin|last4=Millenson|first4=Michael M.|last5=Smith|first5=Mitchell R.|date=2010-01|title=Pediatric nodal marginal zone lymphoma may develop in the adult population|url=https://pubmed.ncbi.nlm.nih.gov/19863176|journal=Leukemia & Lymphoma|volume=51|issue=1|pages=89–94|doi=10.3109/10428190903349670|issn=1029-2403|pmc=3572776|pmid=19863176}}</ref>
* <2% of non-Hodgkin lymphoma<ref name=":5" /><ref name=":12" />
==Clinical Features==
Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table. Do not delete table.'') </span>
{| class="wikitable"
|'''Signs and Symptoms'''
|<span class="blue-text">EXAMPLE:</span> Asymptomatic (incidental finding on complete blood counts)
<span class="blue-text">EXAMPLE:</span> B-symptoms (weight loss, fever, night sweats)
<span class="blue-text">EXAMPLE:</span> Fatigue
<span class="blue-text">EXAMPLE:</span> Lymphadenopathy (uncommon)
|-
|'''Laboratory Findings'''
|<span class="blue-text">EXAMPLE:</span> Cytopenias
<span class="blue-text">EXAMPLE:</span> Lymphocytosis (low level)
|}
<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Features|The content below was from the old template. Please incorporate above.}}</blockquote>
* Asymptomatic in most<ref name=":6" /><ref name=":8" /><ref name=":11" /><ref name=":12" />
* Peripheral lymphadenopathy<ref name=":6" /><ref name=":8" /><ref name=":11" /><ref name=":12">{{Cite journal|last=Attarbaschi|first=Andishe|last2=Abla|first2=Oussama|last3=Arias Padilla|first3=Laura|last4=Beishuizen|first4=Auke|last5=Burke|first5=G. A. Amos|last6=Brugières|first6=Laurence|last7=Bruneau|first7=Julie|last8=Burkhardt|first8=Birgit|last9=d'Amore|first9=Emanuele S. G.|date=08 2020|title=Rare non-Hodgkin lymphoma of childhood and adolescence: A consensus diagnostic and therapeutic approach to pediatric-type follicular lymphoma, marginal zone lymphoma, and nonanaplastic peripheral T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/32452165|journal=Pediatric Blood & Cancer|volume=67|issue=8|pages=e28416|doi=10.1002/pbc.28416|issn=1545-5017|pmid=32452165}}</ref>
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<center><span style="color:Maroon">'''End of V4 Section'''</span>
----
</blockquote>
==Sites of Involvement==
* Primarily head and neck lymph nodes<ref name=":3" /><ref name=":5" /><ref name=":6" /><ref name=":0" /><ref name=":7" /><ref name=":8" /><ref name=":9" />
==Morphologic Features==
*
* Effacement of lymph node architecture due to expansion of marginal zone and intrafollicular proliferation
** Expanded marginal zone may be delineated by IgD staining<ref name=":6" /><ref name=":8" />
* Follicular hyperplasia with features of progressive transformation of germinal centers (PTGC)<ref name=":3" /><ref name=":6" /><ref name=":8" /><ref name=":7" />
** May distinguish pNMZL from adult-type [[HAEM5:Nodal marginal zone lymphoma|NMZL]] and nodal [[HAEM5:Paediatric-type follicular lymphoma|paediatric-type follicular lymphoma]]<ref name=":3" /><ref name=":7" />
* Polymorphic infiltrate composed of small- to medium-sized cells with round nuclei and moderate cytoplasm<ref name=":3" /><ref name=":8" />
* Starry-sky appearance of residual hyperplastic germinal centers<ref name=":7" />
==Immunophenotype==
* Similar to adult-type NMZL, pNMZL is almost universally positive for the mature B cell marker CD20 (90-100%) with most cases also expressing the pan-T cell marker CD43 (70-100%)<ref name=":3" /><ref name=":6" /><ref name=":8" /><ref name=":7" /><ref name=":10">{{Cite journal|last=Elenitoba-Johnson|first=K. S.|last2=Kumar|first2=S.|last3=Lim|first3=M. S.|last4=Kingma|first4=D. W.|last5=Raffeld|first5=M.|last6=Jaffe|first6=E. S.|date=1997-01|title=Marginal zone B-cell lymphoma with monocytoid B-cell lymphocytes in pediatric patients without immunodeficiency. A report of two cases|url=https://pubmed.ncbi.nlm.nih.gov/8980374|journal=American Journal of Clinical Pathology|volume=107|issue=1|pages=92–98|doi=10.1093/ajcp/107.1.92|issn=0002-9173|pmid=8980374}}</ref>
* A subset of pNMZL express BCL2 (40-50%) and IgD (20-30%)<ref name=":3" /><ref name=":6" /><ref name=":8" />
* pNMZL cells are negative for the germinal center markers CD10, BCL6, CD23, and the T cell markers CD3, CD5<ref name=":3" /><ref name=":6" /><ref name=":8" /><ref name=":9" />
* CD279/PD-1 staining present in reactive germinal centers of pNMZL, compared to positive staining at the periphery of germinal centers in nodal [[HAEM5:Paediatric-type follicular lymphoma|pediatric-type follicular lymphoma]]<ref name=":7" />
{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (universal)||CD19, CD20, sIg (bright, monoclonal), CD43
|-
|Positive (subset)||BCL2, CD279/PD-1, IgD
|-
|Negative (universal)||CD10, BCL6, CD23, CD5, CD3, LEF1
|}
|}


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* No chromosomal rearrangements or gene fusions associated with pNMZL<ref name=":0" />
*No chromosomal rearrangements or gene fusions associated with pNMZL<ref name=":0">{{Cite journal|last=Ozawa|first=Michael G.|last2=Bhaduri|first2=Aparna|last3=Chisholm|first3=Karen M.|last4=Baker|first4=Steven A.|last5=Ma|first5=Lisa|last6=Zehnder|first6=James L.|last7=Luna-Fineman|first7=Sandra|last8=Link|first8=Michael P.|last9=Merker|first9=Jason D.|date=10 2016|title=A study of the mutational landscape of pediatric-type follicular lymphoma and pediatric nodal marginal zone lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/27338637|journal=Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc|volume=29|issue=10|pages=1212–1220|doi=10.1038/modpathol.2016.102|issn=1530-0285|pmc=5047957|pmid=27338637}}</ref>
* Clonal rearrangements of immunoglobulin (Ig) region detected in most cases<ref name=":3" /><ref name=":9" />
*Clonal rearrangements of immunoglobulin (Ig) region detected in most cases<ref name=":3">{{Cite journal|last=Koo|first=Matthew|last2=Ohgami|first2=Robert S.|date=2017-05|title=Pediatric-type Follicular Lymphoma and Pediatric Nodal Marginal Zone Lymphoma: Recent Clinical, Morphologic, Immunophenotypic, and Genetic Insights|url=https://pubmed.ncbi.nlm.nih.gov/28277421|journal=Advances in Anatomic Pathology|volume=24|issue=3|pages=128–135|doi=10.1097/PAP.0000000000000144|issn=1533-4031|pmid=28277421}}</ref><ref name=":9">{{Cite journal|displayauthors=1|last=Rizzo|first=Kathryn|date=|title=Marginal zone lymphomas in children and the young adult population; characterization of genetic aberrations by FISH and RT-PCR|url=|journal=Mol Pathol|volume=23|pages=866-873|doi=10.1038/modpathol.2010.63|pmc=PMC6329460|pmid=20305621|via=}}</ref>


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


* No genomic findings currently assist in diagnosis, prognostication, or therapeutic decisions
*No genomic findings currently assist in diagnosis, prognostication, or therapeutic decisions


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* Trisomy 3 and 18 are infrequently described chromosomal aberrations reported in pNMZL<ref name=":9" />
*Trisomy 3 and 18 are infrequently described chromosomal aberrations reported in pNMZL<ref name=":9" />
** These chromosome gains have also been described in adult-type NMZL<ref name=":2" /><ref>{{Cite journal|last=Rinaldi|first=Andrea|last2=Mian|first2=Michael|last3=Chigrinova|first3=Ekaterina|last4=Arcaini|first4=Luca|last5=Bhagat|first5=Govind|last6=Novak|first6=Urban|last7=Rancoita|first7=Paola M. V.|last8=De Campos|first8=Cassio P.|last9=Forconi|first9=Francesco|date=2011-02-03|title=Genome-wide DNA profiling of marginal zone lymphomas identifies subtype-specific lesions with an impact on the clinical outcome|url=https://pubmed.ncbi.nlm.nih.gov/21115979|journal=Blood|volume=117|issue=5|pages=1595–1604|doi=10.1182/blood-2010-01-264275|issn=1528-0020|pmid=21115979}}</ref><ref>{{Cite journal|last=Dierlamm|first=J.|last2=Pittaluga|first2=S.|last3=Wlodarska|first3=I.|last4=Stul|first4=M.|last5=Thomas|first5=J.|last6=Boogaerts|first6=M.|last7=Michaux|first7=L.|last8=Driessen|first8=A.|last9=Mecucci|first9=C.|date=1996-01-01|title=Marginal zone B-cell lymphomas of different sites share similar cytogenetic and morphologic features|url=https://pubmed.ncbi.nlm.nih.gov/8547655|journal=Blood|volume=87|issue=1|pages=299–307|issn=0006-4971|pmid=8547655}}</ref>
**These chromosome gains have also been described in adult-type NMZL<ref name=":2">Swerdlow SH, Campo E, Harris NL et al (eds) WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. IARC Press, Lyon, pp 263-264</ref><ref>{{Cite journal|last=Rinaldi|first=Andrea|last2=Mian|first2=Michael|last3=Chigrinova|first3=Ekaterina|last4=Arcaini|first4=Luca|last5=Bhagat|first5=Govind|last6=Novak|first6=Urban|last7=Rancoita|first7=Paola M. V.|last8=De Campos|first8=Cassio P.|last9=Forconi|first9=Francesco|date=2011-02-03|title=Genome-wide DNA profiling of marginal zone lymphomas identifies subtype-specific lesions with an impact on the clinical outcome|url=https://pubmed.ncbi.nlm.nih.gov/21115979|journal=Blood|volume=117|issue=5|pages=1595–1604|doi=10.1182/blood-2010-01-264275|issn=1528-0020|pmid=21115979}}</ref><ref>{{Cite journal|last=Dierlamm|first=J.|last2=Pittaluga|first2=S.|last3=Wlodarska|first3=I.|last4=Stul|first4=M.|last5=Thomas|first5=J.|last6=Boogaerts|first6=M.|last7=Michaux|first7=L.|last8=Driessen|first8=A.|last9=Mecucci|first9=C.|date=1996-01-01|title=Marginal zone B-cell lymphomas of different sites share similar cytogenetic and morphologic features|url=https://pubmed.ncbi.nlm.nih.gov/8547655|journal=Blood|volume=87|issue=1|pages=299–307|issn=0006-4971|pmid=8547655}}</ref>


{| class="wikitable sortable"
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|Whole chromosome
|Whole chromosome
|0-5%
|0-5%
|<ref name=":10" />
|<ref name=":10">{{Cite journal|last=Elenitoba-Johnson|first=K. S.|last2=Kumar|first2=S.|last3=Lim|first3=M. S.|last4=Kingma|first4=D. W.|last5=Raffeld|first5=M.|last6=Jaffe|first6=E. S.|date=1997-01|title=Marginal zone B-cell lymphoma with monocytoid B-cell lymphocytes in pediatric patients without immunodeficiency. A report of two cases|url=https://pubmed.ncbi.nlm.nih.gov/8980374|journal=American Journal of Clinical Pathology|volume=107|issue=1|pages=92–98|doi=10.1093/ajcp/107.1.92|issn=0002-9173|pmid=8980374}}</ref>
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* No characteristic chromosomal aberrations or patterns reported
*No characteristic chromosomal aberrations or patterns reported


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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.


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* Genes reported to be altered in adult-type NMZL, including ''MLL2 (KMT2D), PTPRD, NOTCH2, KLF2,'' and ''BRAF'',<ref>{{Cite journal|last=Spina|first=Valeria|last2=Khiabanian|first2=Hossein|last3=Messina|first3=Monica|last4=Monti|first4=Sara|last5=Cascione|first5=Luciano|last6=Bruscaggin|first6=Alessio|last7=Spaccarotella|first7=Elisa|last8=Holmes|first8=Antony B.|last9=Arcaini|first9=Luca|date=09 08, 2016|title=The genetics of nodal marginal zone lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/27335277|journal=Blood|volume=128|issue=10|pages=1362–1373|doi=10.1182/blood-2016-02-696757|issn=1528-0020|pmc=5016706|pmid=27335277}}</ref><ref>{{Cite journal|last=Pillonel|first=V.|last2=Juskevicius|first2=D.|last3=Ng|first3=C. K. Y.|last4=Bodmer|first4=A.|last5=Zettl|first5=A.|last6=Jucker|first6=D.|last7=Dirnhofer|first7=S.|last8=Tzankov|first8=A.|date=11 2018|title=High-throughput sequencing of nodal marginal zone lymphomas identifies recurrent BRAF mutations|url=https://pubmed.ncbi.nlm.nih.gov/29556019|journal=Leukemia|volume=32|issue=11|pages=2412–2426|doi=10.1038/s41375-018-0082-4|issn=1476-5551|pmc=6224405|pmid=29556019}}</ref> have not been described in pNMZL<ref name=":0" />
*Genes reported to be altered in adult-type NMZL, including ''MLL2 (KMT2D), PTPRD, NOTCH2, KLF2,'' and ''BRAF'',<ref>{{Cite journal|last=Spina|first=Valeria|last2=Khiabanian|first2=Hossein|last3=Messina|first3=Monica|last4=Monti|first4=Sara|last5=Cascione|first5=Luciano|last6=Bruscaggin|first6=Alessio|last7=Spaccarotella|first7=Elisa|last8=Holmes|first8=Antony B.|last9=Arcaini|first9=Luca|date=09 08, 2016|title=The genetics of nodal marginal zone lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/27335277|journal=Blood|volume=128|issue=10|pages=1362–1373|doi=10.1182/blood-2016-02-696757|issn=1528-0020|pmc=5016706|pmid=27335277}}</ref><ref>{{Cite journal|last=Pillonel|first=V.|last2=Juskevicius|first2=D.|last3=Ng|first3=C. K. Y.|last4=Bodmer|first4=A.|last5=Zettl|first5=A.|last6=Jucker|first6=D.|last7=Dirnhofer|first7=S.|last8=Tzankov|first8=A.|date=11 2018|title=High-throughput sequencing of nodal marginal zone lymphomas identifies recurrent BRAF mutations|url=https://pubmed.ncbi.nlm.nih.gov/29556019|journal=Leukemia|volume=32|issue=11|pages=2412–2426|doi=10.1038/s41375-018-0082-4|issn=1476-5551|pmc=6224405|pmid=29556019}}</ref> have not been described in pNMZL<ref name=":0" />
* While no recurrent somatic variations have been identified in pNMZL, a somatic variant in ''AMOTL1'' (NM_130847, p.Ala891Thr) was reported in a single individual with pNMZL<ref name=":0" />
*While no recurrent somatic variations have been identified in pNMZL, a somatic variant in ''AMOTL1'' (NM_130847, p.Ala891Thr) was reported in a single individual with pNMZL<ref name=":0" />
** Missense alterations in ''AMOTL1'' have been reported in adult splenic marginal zone lymphoma (p.Ala424Thr and p.Val361Ile)<ref>{{Cite journal|last=Parry|first=Marina|last2=Rose-Zerilli|first2=Matthew J. J.|last3=Gibson|first3=Jane|last4=Ennis|first4=Sarah|last5=Walewska|first5=Renata|last6=Forster|first6=Jade|last7=Parker|first7=Helen|last8=Davis|first8=Zadie|last9=Gardiner|first9=Anne|date=2013|title=Whole exome sequencing identifies novel recurrently mutated genes in patients with splenic marginal zone lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/24349473|journal=PloS One|volume=8|issue=12|pages=e83244|doi=10.1371/journal.pone.0083244|issn=1932-6203|pmc=3862727|pmid=24349473}}</ref><ref>{{Cite journal|last=Rossi|first=Davide|last2=Trifonov|first2=Vladimir|last3=Fangazio|first3=Marco|last4=Bruscaggin|first4=Alessio|last5=Rasi|first5=Silvia|last6=Spina|first6=Valeria|last7=Monti|first7=Sara|last8=Vaisitti|first8=Tiziana|last9=Arruga|first9=Francesca|date=2012-08-27|title=The coding genome of splenic marginal zone lymphoma: activation of NOTCH2 and other pathways regulating marginal zone development|url=https://pubmed.ncbi.nlm.nih.gov/22891273|journal=The Journal of Experimental Medicine|volume=209|issue=9|pages=1537–1551|doi=10.1084/jem.20120904|issn=1540-9538|pmc=3428941|pmid=22891273}}</ref> and other cancers ([https://cancer.sanger.ac.uk/cosmic/mutation/overview?id=122769838 COSMIC], [http://www.cbioportal.org/ cBioPortal], [https://pedcbioportal.kidsfirstdrc.org/ PedcBioPortal])
**Missense alterations in ''AMOTL1'' have been reported in adult splenic marginal zone lymphoma (p.Ala424Thr and p.Val361Ile)<ref>{{Cite journal|last=Parry|first=Marina|last2=Rose-Zerilli|first2=Matthew J. J.|last3=Gibson|first3=Jane|last4=Ennis|first4=Sarah|last5=Walewska|first5=Renata|last6=Forster|first6=Jade|last7=Parker|first7=Helen|last8=Davis|first8=Zadie|last9=Gardiner|first9=Anne|date=2013|title=Whole exome sequencing identifies novel recurrently mutated genes in patients with splenic marginal zone lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/24349473|journal=PloS One|volume=8|issue=12|pages=e83244|doi=10.1371/journal.pone.0083244|issn=1932-6203|pmc=3862727|pmid=24349473}}</ref><ref>{{Cite journal|last=Rossi|first=Davide|last2=Trifonov|first2=Vladimir|last3=Fangazio|first3=Marco|last4=Bruscaggin|first4=Alessio|last5=Rasi|first5=Silvia|last6=Spina|first6=Valeria|last7=Monti|first7=Sara|last8=Vaisitti|first8=Tiziana|last9=Arruga|first9=Francesca|date=2012-08-27|title=The coding genome of splenic marginal zone lymphoma: activation of NOTCH2 and other pathways regulating marginal zone development|url=https://pubmed.ncbi.nlm.nih.gov/22891273|journal=The Journal of Experimental Medicine|volume=209|issue=9|pages=1537–1551|doi=10.1084/jem.20120904|issn=1540-9538|pmc=3428941|pmid=22891273}}</ref> and other cancers ([https://cancer.sanger.ac.uk/cosmic/mutation/overview?id=122769838 COSMIC], [http://www.cbioportal.org/ cBioPortal], [https://pedcbioportal.kidsfirstdrc.org/ PedcBioPortal])


{| class="wikitable sortable"
{| class="wikitable sortable"
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===Other Mutations===
===Other Mutations===


* Additional studies are needed to assess the spectrum of somatic variation in pNMZL. A single report evaluated the genetic landscape of pNMZL by whole exome sequencing (n=4) identified missense changes in the following genes: ''AMOTL1, SCAF1, SELPLG, FAM5B, KLHDC4, RAX, PEG3, CHPF, ACTRT3, NRCAM, CHMP1A, CISH, TTC17, NLE1<ref name=":0" />''
*Additional studies are needed to assess the spectrum of somatic variation in pNMZL. A single report evaluated the genetic landscape of pNMZL by whole exome sequencing (n=4) identified missense changes in the following genes: ''AMOTL1, SCAF1, SELPLG, FAM5B, KLHDC4, RAX, PEG3, CHPF, ACTRT3, NRCAM, CHMP1A, CISH, TTC17, NLE1<ref name=":0" />''


<blockquote class="blockedit">
<blockquote class="blockedit">
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==Epigenomic Alterations==
==Epigenomic Alterations==


* None
*None


==Genes and Main Pathways Involved==
==Genes and Main Pathways Involved==
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|}
|}


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


* ''AMOTL1'' encodes angiomotin like-1 which associates with tight junctions and regulates the Hippo signaling pathway<ref>{{Cite journal|last=Yi|first=Chunling|last2=Troutman|first2=Scott|last3=Fera|first3=Daniela|last4=Stemmer-Rachamimov|first4=Anat|last5=Avila|first5=Jacqueline L.|last6=Christian|first6=Neepa|last7=Persson|first7=Nathalie Luna|last8=Shimono|first8=Akihiko|last9=Speicher|first9=David W.|date=2011-04-12|title=A tight junction-associated Merlin-angiomotin complex mediates Merlin's regulation of mitogenic signaling and tumor suppressive functions|url=https://pubmed.ncbi.nlm.nih.gov/21481793|journal=Cancer Cell|volume=19|issue=4|pages=527–540|doi=10.1016/j.ccr.2011.02.017|issn=1878-3686|pmc=3075552|pmid=21481793}}</ref><ref>{{Cite journal|last=Lv|first=Meng|last2=Shen|first2=Yanwei|last3=Yang|first3=Jiao|last4=Li|first4=Shuting|last5=Wang|first5=Biyuan|last6=Chen|first6=Zheling|last7=Li|first7=Pan|last8=Liu|first8=Peijun|last9=Yang|first9=Jin|date=2017|title=Angiomotin Family Members: Oncogenes or Tumor Suppressors?|url=https://pubmed.ncbi.nlm.nih.gov/28656002|journal=International Journal of Biological Sciences|volume=13|issue=6|pages=772–781|doi=10.7150/ijbs.19603|issn=1449-2288|pmc=5485632|pmid=28656002}}</ref>
*''AMOTL1'' encodes angiomotin like-1 which associates with tight junctions and regulates the Hippo signaling pathway<ref>{{Cite journal|last=Yi|first=Chunling|last2=Troutman|first2=Scott|last3=Fera|first3=Daniela|last4=Stemmer-Rachamimov|first4=Anat|last5=Avila|first5=Jacqueline L.|last6=Christian|first6=Neepa|last7=Persson|first7=Nathalie Luna|last8=Shimono|first8=Akihiko|last9=Speicher|first9=David W.|date=2011-04-12|title=A tight junction-associated Merlin-angiomotin complex mediates Merlin's regulation of mitogenic signaling and tumor suppressive functions|url=https://pubmed.ncbi.nlm.nih.gov/21481793|journal=Cancer Cell|volume=19|issue=4|pages=527–540|doi=10.1016/j.ccr.2011.02.017|issn=1878-3686|pmc=3075552|pmid=21481793}}</ref><ref>{{Cite journal|last=Lv|first=Meng|last2=Shen|first2=Yanwei|last3=Yang|first3=Jiao|last4=Li|first4=Shuting|last5=Wang|first5=Biyuan|last6=Chen|first6=Zheling|last7=Li|first7=Pan|last8=Liu|first8=Peijun|last9=Yang|first9=Jin|date=2017|title=Angiomotin Family Members: Oncogenes or Tumor Suppressors?|url=https://pubmed.ncbi.nlm.nih.gov/28656002|journal=International Journal of Biological Sciences|volume=13|issue=6|pages=772–781|doi=10.7150/ijbs.19603|issn=1449-2288|pmc=5485632|pmid=28656002}}</ref>


<blockquote class="blockedit">
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==Genetic Diagnostic Testing Methods==
==Genetic Diagnostic Testing Methods==


* Histopathology and immunophenotyping
*Histopathology and immunophenotyping
* Molecular testing (i.e. clonality assessment)
*Molecular testing (i.e. clonality assessment)


==Familial Forms==
==Familial Forms==


* Not described
*Not described


==Additional Information==
==Additional Information==


* None
*None


==Links==
==Links==


* [[HAEM5:Nodal marginal zone lymphoma]]
*[[HAEM5:Nodal marginal zone lymphoma]]
* [[HAEM5:Paediatric-type follicular lymphoma]]
*[[HAEM5:Paediatric-type follicular lymphoma]]


==References==
==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 />
(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 />


'''
<br />


==Notes==
==Notes==
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<nowiki>*</nowiki>''Citation of this Page'': “Paediatric nodal marginal zone lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Paediatric_nodal_marginal_zone_lymphoma</nowiki>.
<nowiki>*</nowiki>''Citation of this Page'': “Paediatric nodal marginal zone lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Paediatric_nodal_marginal_zone_lymphoma</nowiki>.
[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases P]]
[[Category:HAEM5]]
[[Category:DISEASE]]
[[Category:Diseases P]]
Retrieved from "https://test.ccga.io/index.php/HAEM5:Paediatric_nodal_marginal_zone_lymphoma"