Bailey.Glen: Created page with "{{Under Construction}} ==Primary Author(s)== Andrew Ly, DO and Shivani Golem, PhD, FACMG TOC ==Cancer Category/Type== Mature B-Cell Neoplasms ==Cancer Sub-Class..."

2023-11-03T17:42:56Z

Created page with "{{Under Construction}} ==Primary Author(s)*== Andrew Ly, DO and Shivani Golem, PhD, FACMG __TOC__ ==Cancer Category/Type== *Mature B-Cell Neoplasms ==Cancer Sub-Class..."

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{{Under Construction}}
==Primary Author(s)*==

Andrew Ly, DO and Shivani Golem, PhD, FACMG

__TOC__

==Cancer Category/Type==

*[[Mature B-Cell Neoplasms]]

==Cancer Sub-Classification / Subtype==

*Nodal Marginal Zone Lymphoma

==Definition / Description of Disease==

Nodal marginal zone lymphoma (NMZL) is an uncommon subtype of non-Hodgkin lymphoma. It is a primary nodal B-cell lymphoma with histological features similar to [[Splenic Marginal Zone Lymphoma|Splenic marginal zone lymphoma]] and [[Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)|Extranodal marginal zone lymphoma]] involving lymph nodes, but without evidence of splenic or extranodal disease<ref name=":0">Campo E, et al., (2017). Nodal marginal zone lymphoma, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p263-264.</ref>.

==Synonyms / Terminology<ref name=":0" />==

*Monocytoid B-cell lymphoma
*Parafollicular B-cell lymphoma (no longer in use)

==Epidemiology / Prevalence<ref name=":0" /><ref>{{Cite journal|last=Arcaini|first=Luca|last2=Paulli|first2=Marco|last3=Burcheri|first3=Sara|last4=Rossi|first4=Andrea|last5=Spina|first5=Michele|last6=Passamonti|first6=Francesco|last7=Lucioni|first7=Marco|last8=Motta|first8=Teresio|last9=Canzonieri|first9=Vincenzo|date=2007-01|title=Primary nodal marginal zone B-cell lymphoma: clinical features and prognostic assessment of a rare disease|url=http://doi.wiley.com/10.1111/j.1365-2141.2006.06437.x|journal=British Journal of Haematology|language=en|volume=136|issue=2|pages=301–304|doi=10.1111/j.1365-2141.2006.06437.x|issn=0007-1048}}</ref><ref>{{Cite journal|last=Brand|first=Michiel van den|last2=Krieken|first2=J. Han J. M. van|date=2013-07-01|title=Recognizing nodal marginal zone lymphoma: recent advances and pitfalls. A systematic review|url=https://haematologica.org/article/view/6708|journal=Haematologica|language=en|volume=98|issue=7|pages=1003–1013|doi=10.3324/haematol.2012.083386|issn=1592-8721|pmc=PMC3696602|pmid=23813646}}</ref>==

*1.5-1.8% of all lymphoid neoplasms
*Median age ~60 years old
*Both sexes are affected equally
*Cases also occur in children and are separately diagnosed as [[Paediatric Nodal Marginal Zone Lymphoma]]
*Association with autoimmune diseases
*Association with Hepatitis C virus infection reported in some studies but not all studies

==Clinical Features==

*Asymptomatic, localized or generalized lymphadenopathy
*B symptoms (fever, night sweats, and weight loss)
*Bone marrow involvement

The presence of a primary extranodal marginal zone lymphoma should be ruled out due to the possibility of a nodal dissemination of a MALT lymphoma occurring in patients with a history of Sjogren syndrome and Hashimoto thyroiditis<ref name=":0" />.

==Sites of Involvement==

*Lymph nodes
*Bone marrow
*Peripheral blood

==Morphologic Features<ref name=":0" />==

*Variable populations of lymphoma cells
*#Centrocyte-like and monocytoid B-cells
*#Plasma cells
*#Scattered transformed B cells
*Lymph nodes show small lymphoma cells surrounding reactive follicles (marginal zone distribution)
*#Extension to interfollicular areas and follicular colonization may be present
*#Diffuse or partial nodal effacement may be present
*Bone marrow shows lymphoma cells in interstitial, nodular, intertrabecular or paratrabecular distribution

==Immunophenotype==
{| class="wikitable"
!Finding
!Marker
|-
|Positive (B-cell lineage markers)
|CD19, CD20, CD22, PAX5, FMC7, CD79a, sIg
|-
|Positive (most cases)
|BCL2, MNDA, IRTA1
|-
|Variable positivity
|CD5, CD43, CD23
|-
|Negative
|CD10, Cyclin D1, BCL6, LMO2
|}

==Chromosomal Rearrangements (Gene Fusions)==

*Recurrent chromosomal translocations that are frequent in other lymphoid malignancies and associated with extranodal MZL are not detected<ref name=":0" /><ref name=":2">{{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=2018-11|title=High-throughput sequencing of nodal marginal zone lymphomas identifies recurrent BRAF mutations|url=http://www.nature.com/articles/s41375-018-0082-4|journal=Leukemia|language=en|volume=32|issue=11|pages=2412–2426|doi=10.1038/s41375-018-0082-4|issn=0887-6924|pmc=PMC6224405|pmid=29556019}}</ref>.

==Characteristic Chromosomal Aberrations / Patterns<ref name=":0" /><ref name=":2" />==

*Deletions in 7q31

==Genomic Gain/Loss/LOH<ref name=":0" />==

{| class="wikitable sortable"
|-
!Chromosome Number!!Gain/Loss/Amp/LOH!!Region
|-
|3||Gain||N/A
|-
|12
|Gain
|N/A
|-
|18||Gain||N/A
|-
|6
|Loss
|6q23-24
|}

==Gene Mutations (SNV/INDEL)==

Whole-exome sequencing (WES) study have identified mutations involved in NOTCH, nuclear factor κB (NF-κB), B-cell receptors and toll like receptor pathways . In one study, 16% (4/25) of cases identified a BRAF (V600E) mutation with associated strong IgD expression. In one of the four BRAF positive mutation, two non-hotspot mutations were detected (L597Q and N581I) which was previously found in BRAF V600 wild-type melanoma. In the same study, mutations of KMT2D (7/25, 28%), TET2 (5/25, 20%), and EZH2 (5/25, 20%) were among the more frequent mutated genes. CREBBP, TNFRSF14, FAS, TNFAIP3, KLF2, and CXCR4 mutations were also detected<ref name=":2" />. In another study, which investigated genetic lesions in 35 patients with NMZL, PTPRD mutations were found in 14.3% (5/35) of patients and PTPRD locus deletions were found in 5.7% (2/35) of patients<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=2016-09-08|title=The genetics of nodal marginal zone lymphoma|url=https://ashpublications.org/blood/article/128/10/1362/35315/The-genetics-of-nodal-marginal-zone-lymphoma|journal=Blood|language=en|volume=128|issue=10|pages=1362–1373|doi=10.1182/blood-2016-02-696757|issn=0006-4971|pmc=PMC5016706|pmid=27335277}}</ref>. Mutations were also identified in another study for NFKBIE and ITPR2 mutations involved in the NF-κB pathway and B-cell receptor mediated calcium signal pathway. However, in this study they did not find any PTPRD mutations or BRAF mutations, demonstrating the diverseness of the disease<ref>{{Cite journal|last=Koh|first=Jiwon|last2=Jang|first2=Insoon|last3=Choi|first3=Seongmin|last4=Kim|first4=Sehui|last5=Jang|first5=Ingeon|last6=Ahn|first6=Hyun Kyung|last7=Lee|first7=Cheol|last8=Paik|first8=Jin Ho|last9=Kim|first9=Chul Woo|date=2020-06-23|title=Discovery of Novel Recurrent Mutations and Clinically Meaningful Subgroups in Nodal Marginal Zone Lymphoma|url=https://www.mdpi.com/2072-6694/12/6/1669|journal=Cancers|language=en|volume=12|issue=6|pages=1669|doi=10.3390/cancers12061669|issn=2072-6694|pmc=PMC7352856|pmid=32585984}}</ref>. No BRAF mutations have yet to be identified in other studies on NMZL<ref name=":2" />.
===Other Mutations===
Immunoglobulin genes are clonally rearranged consisting of mutated IGHV3 and IGHV4 family members, particularly IGHV4-34 and cases associated with hepatitis C use IGHV1-69<ref name=":0" />.

==Epigenomics (Methylation)==

*Not known in this specific subtype.

==Genes and Main Pathways Involved==

*NF-κB pathway and B-cell receptor mediated calcium signal pathway.

==Diagnostic Testing Methods==

*No diagnostic test is specifically established.

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

*None.

==Familial Forms==

*Not known in this specific subtype.

==Other Information==

None

==Links==

*[[Paediatric Nodal Marginal Zone Lymphoma]]
*[[Splenic Marginal Zone Lymphoma]]
*[[Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)|Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue]]

==References==
<references />

==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>The hierarchical tumour classification structure displayed on this page is reproduced from the [https://tumourclassification.iarc.who.int/welcome/ WHO Classification of Tumours] with permission from the copyright holder, ©International Agency for Research on Cancer.

HAEM4Backup:Nodal Marginal Zone Lymphoma - Revision history

Bailey.Glen: Created page with "{{Under Construction}} ==Primary Author(s)*== Andrew Ly, DO and Shivani Golem, PhD, FACMG __TOC__ ==Cancer Category/Type== *Mature B-Cell Neoplasms ==Cancer Sub-Class..."

Bailey.Glen: Created page with "{{Under Construction}} ==Primary Author(s)== Andrew Ly, DO and Shivani Golem, PhD, FACMG TOC ==Cancer Category/Type== Mature B-Cell Neoplasms ==Cancer Sub-Class..."