HAEM5:Follicular dendritic cell sarcoma: Difference between revisions

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{{DISPLAYTITLE:Follicular dendritic cell sarcoma}}
{{DISPLAYTITLE:Follicular dendritic cell sarcoma}}
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]


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<blockquote class='blockedit'>{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Follicular Dendritic Cell Sarcoma]].
<blockquote class="blockedit">{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Follicular Dendritic Cell Sarcoma]].
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==Definition / Description of Disease==
Follicular dendritic cell sarcoma (FDCS) is a neoplasm with morphologic and immunophenotypic features of follicular dendritic cells, which are normally present in lymphoid follicles and are derived from mesenchymal cells of lymphoid tissues.
==Synonyms / Terminology==
Follicular dendritic cell tumor
Dendritic reticulum cell tumor (no longer used)
==Epidemiology / Prevalence==
Put your text here
==Clinical Features==
Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table. Do not delete table.'') </span>
{| class="wikitable"
|'''Signs and Symptoms'''
|<span class="blue-text">EXAMPLE:</span> Asymptomatic (incidental finding on complete blood counts)
<span class="blue-text">EXAMPLE:</span> B-symptoms (weight loss, fever, night sweats)
<span class="blue-text">EXAMPLE:</span> Fatigue
<span class="blue-text">EXAMPLE:</span> Lymphadenopathy (uncommon)
|-
|'''Laboratory Findings'''
|<span class="blue-text">EXAMPLE:</span> Cytopenias
<span class="blue-text">EXAMPLE:</span> Lymphocytosis (low level)
|}
==Sites of Involvement==
*Extranodal sites (79%)
**Liver, spleen, and GI tract most common
**Any site may be involved
*Lymph nodes (~15%)<ref name=":2">{{Cite journal|last=Facchetti|first=Fabio|last2=Simbeni|first2=Matteo|last3=Lorenzi|first3=Luisa|date=2021-10|title=Follicular dendritic cell sarcoma|url=https://pubmed.ncbi.nlm.nih.gov/34837090|journal=Pathologica|volume=113|issue=5|pages=316–329|doi=10.32074/1591-951X-331|issn=1591-951X|pmc=8720404|pmid=34837090}}</ref>
==Morphologic Features==
Put your text here
==Immunophenotype==
{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (lineage-defining)||One or more of CD21, CD23, CD35
|-
|Positive (frequent)||CXCL13, clusterin, podoplanin, fascin, vimentin
|-
|Positive (variable)
|CD68, EMA, S100
|-
|Negative (universal)||CD1a, langerin, CD34, CD45, lysozyme, CD163, MPO, CD3, CD79a, cytokeratins, HMB-45
|}
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<center><span style="color:Maroon">'''End of V4 Section'''</span>
----
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==WHO Essential and Desirable Genetic Diagnostic Criteria==
==WHO Essential and Desirable Genetic Diagnostic Criteria==
<span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span>
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The following rearrangements have been reported in individual cases, but whether they are recurrent is not yet known.
The following rearrangements have been reported in individual cases, but whether they are recurrent is not yet known.
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Although no recurrent chromosomal alterations have been identified in FDCS, the tumors often show complex karyotypes with loss of whole or partial chromosomes being the most frequent aberration. Losses frequently occur in regions harboring important tumor suppressor genes.<ref name=":2" />
Although no recurrent chromosomal alterations have been identified in FDCS, the tumors often show complex karyotypes with loss of whole or partial chromosomes being the most frequent aberration. Losses frequently occur in regions harboring important tumor suppressor genes.<ref name=":2">{{Cite journal|last=Facchetti|first=Fabio|last2=Simbeni|first2=Matteo|last3=Lorenzi|first3=Luisa|date=2021-10|title=Follicular dendritic cell sarcoma|url=https://pubmed.ncbi.nlm.nih.gov/34837090|journal=Pathologica|volume=113|issue=5|pages=316–329|doi=10.32074/1591-951X-331|issn=1591-951X|pmc=8720404|pmid=34837090}}</ref>


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|}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.
|}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.


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FDCS primarily shows alterations in the NF-κB signaling pathway. Unlike the dendritic cell and histiocytic neoplasms of hematopoietic origin, aberrations in the MAPK pathway are uncommon.  
FDCS primarily shows alterations in the NF-κB signaling pathway. Unlike the dendritic cell and histiocytic neoplasms of hematopoietic origin, aberrations in the MAPK pathway are uncommon.  
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'''
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==Notes==
==Notes==
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<nowiki>*</nowiki>''Citation of this Page'': “Follicular dendritic cell sarcoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Follicular_dendritic_cell_sarcoma</nowiki>.
<nowiki>*</nowiki>''Citation of this Page'': “Follicular dendritic cell sarcoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Follicular_dendritic_cell_sarcoma</nowiki>.
[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases F]]
[[Category:HAEM5]]
[[Category:DISEASE]]
[[Category:Diseases F]]