Changes

==Cancer Category/Type==

==Cancer Category/Type==

* [[Mature B-Cell Neoplasms]]

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

==Cancer Sub-Classification / Subtype==

==Cancer Sub-Classification / Subtype==

* [[Nodal Marginal Zone Lymphoma]] (NMZL)

* [[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 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>

* 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>

** 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 Marginal Zone Lymphoma|NMZL]] and nodal [[Paediatric-Type Follicular Lymphoma|paediatric-type follicular lymphoma]]<ref name=":3" /><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" />

* 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" />

* 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-Type Follicular Lymphoma|pediatric-type follicular lymphoma]]<ref name=":7" />

* 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"

==Links==

==Links==

* [[Nodal Marginal Zone Lymphoma]]

* [[HAEM5:Nodal marginal zone lymphoma]]

* [[Paediatric-Type Follicular Lymphoma]]

* [[HAEM5:Paediatric-type follicular lymphoma]]

==References==

==References==