HAEM4:Paediatric Nodal Marginal Zone Lymphoma: Difference between revisions
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{{DISPLAYTITLE:Paediatric Nodal Marginal Zone 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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==Cancer Category/Type== | ==Cancer Category/Type== | ||
* [[Mature B-Cell Neoplasms]] | * [[HAEM4:Mature B-Cell Neoplasms]] | ||
==Cancer Sub-Classification / Subtype== | ==Cancer Sub-Classification / Subtype== | ||
* [[Nodal | * [[HAEM5:Nodal marginal zone lymphoma]] (NMZL) | ||
==Definition / Description of Disease== | ==Definition / Description of Disease== | ||
* Paediatric nodal marginal zone lymphoma (pNMZL) is a rare and distinct entity of [[Nodal | * 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> | * 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> | * 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> | ||
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** Expanded marginal zone may be delineated by IgD staining<ref name=":6" /><ref name=":8" /> | ** 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" /> | * 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 [[Nodal | ** 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" /> | * 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" /> | * Starry-sky appearance of residual hyperplastic germinal centers<ref name=":7" /> | ||
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* A subset of pNMZL express BCL2 (40-50%) and IgD (20-30%)<ref name=":3" /><ref name=":6" /><ref name=":8" /> | * 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" /> | * 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 [[Paediatric- | * 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" | {| class="wikitable sortable" | ||
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==Links== | ==Links== | ||
* [[Nodal | * [[HAEM5:Nodal marginal zone lymphoma]] | ||
* [[Paediatric- | * [[HAEM5:Paediatric-type follicular lymphoma]] | ||
==References== | ==References== | ||
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==Notes== | ==Notes== | ||
<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. | <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. | ||
[[Category:HAEM4]] [[Category:DISEASE]] | |||