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Marginal zone lymphoma (view source)

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#redirect [[Small_cell_lymphomas#Marginal_zone_lymphoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Marginal zone lymphoma - kidney -- intermed mag.jpg
| Width      =
| Caption    = Marginal zone lymphoma of the kidney. [[H&E stain]].
| Synonyms  =
| Micro      = small lymphoid cells +/-plasmacytoid features +/-[[lymphoepithelial lesion]]
| Subtypes  =
| LMDDx      = other [[small cell lymphomas]], [[DLBCL]]
| Stains    =
| IHC        = CD20 +ve, BCL2 +ve, CD21 +ve, CD43 +ve/-ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = gastrointestinal tract, [[lymph nodes]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = moderate
| Bloodwork  =
| Rads      =
| Endoscopy  = +/-nodularity
| Prognosis  =
| Other      = CD11c +ve (flow cytometry or laser scanning cytometry)
| ClinDDx    =
| Tx        =
}}
'''Marginal zone lymphoma''', abbreviated '''MZL''', is a common type of lymphoma composed of [[small cell lymphomas|small cells]].
 
==General==
*The MALTomas arise in the context chronic infective or inflammatory conditions, e.g. [[Sjögren disease]] ([[salivary gland]]), [[Hashimoto thyroiditis]] ([[thyroid gland]]), [[Helicobacter pylori gastritis]] ([[stomach]]).<ref name=Ref_PCPBoD8_326>{{Ref PCPBoD8|326}}</ref>
 
===Classification===
*Comes in three different flavours:
*#Extranodal marginal zone lymphoma.
*#*If in ''mucosa-associated lymphoid tissue'' known as a ''MALT lymphoma'', [[AKA]] ''MALToma''. There is also a counterpart in the [[lung]] that arises from ''bronchus-associated lymphoid tissue''. These are known as ''[[BALToma]]s''.
*#Splenic marginal zone lymphoma (SMZL).
*#Nodal marginal zone lymphoma (NMZL).
 
Whilst there are some immunohistochemical differences between the 3 types, separating them out depends on clinical features and radiological findings, e.g. splenic marginal zone lymphoma with spread to lymph nodes may be indistinguishable from primary nodal marginal zone lymphoma.
 
==Microscopic==
Features:
*Small (lymphoid) cells that may be plasma cell-like (plasmacytoid):<ref>URL: [http://surgpathcriteria.stanford.edu/bcell/marginalnodal/printable.html http://surgpathcriteria.stanford.edu/bcell/marginalnodal/printable.html]. Accessed on: 6 March 2012.</ref>
**+/-Clockface nucleus.
**+/-Eccentric nucleus.
*+/-"[[Lymphoepithelial lesion]]" - gastric crypts invaded by a monomorphous population of lymphocytes.<ref name=pmid1452124>{{Cite journal  | last1 = Papadaki | first1 = L. | last2 = Wotherspoon | first2 = AC. | last3 = Isaacson | first3 = PG. | title = The lymphoepithelial lesion of gastric low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT): an ultrastructural study. | journal = Histopathology | volume = 21 | issue = 5 | pages = 415-21 | month = Nov | year = 1992 | doi =  | PMID = 1452124 }}</ref>
**Features:
**# Cluster of lymphocytes - three cells or more - '''key feature'''.
**#* Single lymphocytes don't count.
**# Clearing around the lymphocyte cluster.
**Not specific for MALT lymphoma, i.e. may be seen in other types of lymphoma.<ref>DB. 6 August 2010.</ref>
 
DDx:
*Other [[small cell lymphomas]].
*Marginal zone hyperplasia
*[[DLBCL]] - should be a distinctive region at low power.{{fact}}
 
===Images===
<gallery>
Image: Marginal zone lymphoma - kidney -- low mag.jpg | MZL - low mag. (WC)
Image: Marginal zone lymphoma - kidney -- intermed mag.jpg | MZL - intermed. mag. (WC)
Image: Marginal zone lymphoma - kidney -- high mag.jpg | MZL - high mag. (WC)
Image: Marginal zone lymphoma - kidney -- very high mag.jpg | MZL - very high mag. (WC)
Image: Marginal zone lymphoma - kidney - alt -- very high mag.jpg | MZL - very high mag. (WC)
</gallery>
 
[[File:5 3078322547565 sl 1.png| MALT lymphoma of stomach]]
[[File:5 3078322547565 sl 2.png| MALT lymphoma of stomach]]
[[File:5 3078322547565 sl 3.png| MALT lymphoma of stomach]]
[[File:5 3078322547565 sl 4.png| MALT lymphoma of stomach]]
[[File:5 3078322547565 sl 5.png| MALT lymphoma of stomach]]<br>
Extrazonal marginal zone lymphojma of mucosa-associates lymphoid tissue (MALT lymphoma) in stomach of 64 Year old man, identified in sleeve gastrectomy performed for weight loss. A. Predominantly submucosal blue masses without definite follicles. B. Small round to irregular lymphoid cells surround the most inferior pits. C. Non-neoplastic T cells are seen in abundance, sometime within pit epithelium.  D. Neoplastic B cells mass and surround pits, but do not extensively invade the epithelium; lymphoepithelial lesions were not seen. E. Cancer cells expressed BCL2.  Cancer cells were negative for CD5, CD10, and cyclin D1.
 
www:
*[http://path.upmc.edu/cases/case405.html MALT lymphoma - several images (upmc.edu)].
*[http://www.pathpedia.com/education/eatlas/histopathology/intestine_large/lymphoma_malt-type.aspx MALT lymphoma - several images (pathpedia.com)].
====Lymphoepithelial lesion====
<gallery>
Image:Gastrointestinal_lymphoepithelial_lesion_-_very_high_mag.jpg | Lymphoepithelial lesion - very high mag. (WC)
Image:Gastrointestinal_lymphoepithelial_lesion_-_intermed_mag.jpg | Lymphoepithelial lesion - intermed. mag. (WC)
</gallery>
 
==IHC==
Features:<ref>{{Ref Lester|95}}</ref>
*CD20 +ve.
*BCL2 +ve.
*CD21 +ve.
*CD11c +ve (flow cytometry or laser scanning cytometry - only; not available for paraffin).
*CD43 +ve/-ve.
**Typically positive in ''[[mantle cell lymphoma]].
 
Others:
*CD5 -ve.
*CD10 -ve.
*CD23 -ve.
==Molecular==
There are several associated with MALT lymphoma:<ref name=pmid16950858>{{cite journal |author=Bacon CM, Du MQ, Dogan A |title=Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists |journal=J. Clin. Pathol. |volume=60 |issue=4 |pages=361–72 |year=2007 |month=April |pmid=16950858 |pmc=2001121 |doi=10.1136/jcp.2005.031146 |url=}}</ref>
*t(11;18)(q21;q21) / API2‐MALT1<ref name=Ref_PCPBoD8_170>{{Ref PCPBoD8|170}}</ref> - most common translocation in MALT lymphoma.<ref name=pmid12406890>{{Cite journal  | last1 = Streubel | first1 = B. | last2 = Lamprecht | first2 = A. | last3 = Dierlamm | first3 = J. | last4 = Cerroni | first4 = L. | last5 = Stolte | first5 = M. | last6 = Ott | first6 = G. | last7 = Raderer | first7 = M. | last8 = Chott | first8 = A. | title = T(14;18)(q32;q21) involving IGH and MALT1 is a frequent chromosomal aberration in MALT lymphoma. | journal = Blood | volume = 101 | issue = 6 | pages = 2335-9 | month = Mar | year = 2003 | doi = 10.1182/blood-2002-09-2963 | PMID = 12406890 }}
</ref>
*t(14;18)(q32;q21) / IGH‐MALT1.
**Should not be confused with t(14;18) seen in [[follicular lymphoma]] between IGH-BCL2.<ref name=pmid18359244>{{Cite journal  | last1 = Vitolo | first1 = U. | last2 = Ferreri | first2 = AJ. | last3 = Montoto | first3 = S. | title = Follicular lymphomas. | journal = Crit Rev Oncol Hematol | volume = 66 | issue = 3 | pages = 248-61 | month = Jun | year = 2008 | doi = 10.1016/j.critrevonc.2008.01.014 | PMID = 18359244 }}</ref>
*t(1;14)(p22;q32) / IGH‐BCL10.
 
The MALT1 associated [[translocations]] can be assessed with an [[ISH]] break apart probe for MALT1.
 
==See also==
*[[Small cell lymphomas]].
*[[Lymphoma]].
*[[BALToma]].
 
==References==
{{Reflist|2}}
 
[[Category:Small cell lymphomas]]
Mark
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