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| {{DISPLAYTITLE:Primary cutaneous marginal zone lymphoma}} | | {{DISPLAYTITLE:Primary cutaneous marginal zone lymphoma}} |
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| [[HAEM5:Table_of_Contents|Haematolymphoid Tumours (5th ed.)]] | | [[HAEM5:Table_of_Contents|Haematolymphoid Tumours (5th ed.)]] |
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| {{Under Construction}} | | {{Under Construction}} |
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| + | <span style="color:#0070C0">(General Instructions – The main focus of these pages is the clinically significant genetic alterations in each disease type. Use [https://www.genenames.org/ <u>HUGO-approved gene names and symbols</u>] (italicized when appropriate), [https://varnomen.hgvs.org/ HGVS-based nomenclature for variants], as well as generic names of drugs and testing platforms or assays if applicable. Please complete tables whenever possible and do not delete them (add N/A if not applicable in the table and delete the examples). Please do not delete or alter the section headings. The use of bullet points alongside short blocks of text rather than only large paragraphs is encouraged. Additional instructions below in italicized blue text should not be included in the final page content. Please also see </span><u>[[Author_Instructions]]</u><span style="color:#0070C0"> and [[Frequently Asked Questions (FAQs)|<u>FAQs</u>]] as well as contact your [[Leadership|<u>Associate Editor</u>]] or [mailto:CCGA@cancergenomics.org <u>Technical Support</u>])</span> |
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| ==Primary Author(s)*== | | ==Primary Author(s)*== |
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− | Put your text here<span style="color:#0070C0"> (''Name and affiliation; example:'' Jane Smith, PhD, Institute of Genomics) </span>
| + | Molly Walkenhorst, DO and Shivani Golem, PhD, FACMG |
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| __TOC__ | | __TOC__ |
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| ==Cancer Category / Type== | | ==Cancer Category / Type== |
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| + | * Mature B-cell Neoplasms |
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| ==Cancer Sub-Classification / Subtype== | | ==Cancer Sub-Classification / Subtype== |
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| + | * Marginal Zone Lymphoma |
| + | ** Primary Cutaneous Marginal Zone Lymphoma |
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| ==Definition / Description of Disease== | | ==Definition / Description of Disease== |
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| + | Primary cutaneous marginal zone lymphoma (PCMZL) is a indolent non-Hodgkin lymphoma arising in skin without evidence of extracutaneous disease at the time of diagnosis. The tumor is comprised of small B-cells, plasma cells, and a variable number of reactive T-cells infiltrating the dermis, often forming follicles with reactive germinal centers. |
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| Put your text here <span style="color:#0070C0">(''Instructions: Brief description of approximately one paragraph - include disease context relative to other WHO classification categories referring to the specific WHO book pages, diagnostic criteria if applicable, and differential diagnosis if applicable'') </span> | | Put your text here <span style="color:#0070C0">(''Instructions: Brief description of approximately one paragraph - include disease context relative to other WHO classification categories referring to the specific WHO book pages, diagnostic criteria if applicable, and differential diagnosis if applicable'') </span> |
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| ==Synonyms / Terminology== | | ==Synonyms / Terminology== |
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− | Put your text here <span style="color:#0070C0">(''Instructions: Include currently used terms and major historical ones, adding “(historical)” after the latter.'') </span>
| + | * Primary cutaneous marginal zone lymphoproliferative disorder |
| + | * Primary cutaneous immunocytoma (historical; no longer in use) |
| + | * Primary cutaneous plasmacytoma (historical; no longer in use) |
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| ==Epidemiology / Prevalence== | | ==Epidemiology / Prevalence== |
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− | Put your text here
| + | * 30-40% of all primary cutaneous B-cell lymphomas |
| + | * Predominantly affects adults in the fifth and sixth decades of life |
| + | * Male preponderance |
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| ==Clinical Features== | | ==Clinical Features== |
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| + | * |
| + | * present with multifocal or, less frequently, solitary red or violaceous plaques or nodules |
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| Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table'') </span> | | Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table'') </span> |
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| ==Sites of Involvement== | | ==Sites of Involvement== |
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− | Put your text here <span style="color:#0070C0">(''Instruction: Indicate physical sites; Example: nodal, extranodal, bone marrow'') </span>
| + | * Skin |
| + | ** Most commonly on the trunk and arms |
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| ==Morphologic Features== | | ==Morphologic Features== |
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| <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. |
| <nowiki>*</nowiki>''Citation of this Page'': “Primary cutaneous marginal zone lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Primary_cutaneous_marginal_zone_lymphoma</nowiki>. | | <nowiki>*</nowiki>''Citation of this Page'': “Primary cutaneous marginal zone lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Primary_cutaneous_marginal_zone_lymphoma</nowiki>. |
− | [[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases P]] | + | [[Category:HAEM5]] |
| + | [[Category:DISEASE]] |
| + | [[Category:Diseases P]] |