Follicular dendritic cell sarcoma

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Haematolymphoid Tumours (WHO Classification, 5th ed.)

editHAEM5 Conversion Notes
This page was converted to the new template on 2023-12-07. The original page can be found at HAEM4:Follicular Dendritic Cell Sarcoma.

(General Instructions – The main focus of these pages is the clinically significant genetic alterations in each disease type. Use HUGO-approved gene names and symbols (italicized when appropriate), 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); to add (or move) a row or column to a table, click nearby within the table and select the > symbol that appears to be given options. 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 Author_Instructions and FAQs as well as contact your Associate Editor or Technical Support)

Primary Author(s)*

Anna Heimes Dillon, MD and Shivani Golem, PhD, FACMG

Cancer Category / Type

HAEM4:Histiocytic and Dendritic Cell Neoplasms (WHO 4th ed.)[1]

Stroma-derived neoplasms of lymphoid tissues (WHO 5th ed.)[2]

Cancer Sub-Classification / Subtype

Mesenchymal dendritic cell neoplasms (WHO 5th ed.)[2]

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

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Clinical Features

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Signs and Symptoms EXAMPLE: Asymptomatic (incidental finding on complete blood counts)

EXAMPLE: B-symptoms (weight loss, fever, night sweats)

EXAMPLE: Fatigue

EXAMPLE: Lymphadenopathy (uncommon)

Laboratory Findings EXAMPLE: Cytopenias

EXAMPLE: Lymphocytosis (low level)

Sites of Involvement

  • Extranodal sites (79%)
    • Liver, spleen, and GI tract most common
    • Any site may be involved
  • Lymph nodes (~15%)[3]

Morphologic Features

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Immunophenotype

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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[1][3]

Chromosomal Rearrangements (Gene Fusions)

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Chromosomal Rearrangement Genes in Fusion (5’ or 3’ Segments) Pathogenic Derivative Prevalence Diagnostic Significance (Yes, No or Unknown) Prognostic Significance (Yes, No or Unknown) Therapeutic Significance (Yes, No or Unknown) Notes
EXAMPLE: t(9;22)(q34;q11.2) EXAMPLE: 3'ABL1 / 5'BCR EXAMPLE: der(22) EXAMPLE: 20% (COSMIC)

EXAMPLE: 30% (add reference)

Yes No Yes EXAMPLE:

The t(9;22) is diagnostic of CML in the appropriate morphology and clinical context (add reference). This fusion is responsive to targeted therapy such as Imatinib (Gleevec) (add reference).


editHAEM5 Conversion Notes
Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification

The following rearrangements have been reported in individual cases, but whether they are recurrent is not yet known.

Chromosomal Rearrangement Genes in Fusion (5’ or 3’ Segments) Pathogenic Derivative Prevalence
EXAMPLE: t(9;22)(q34;q11.2) EXAMPLE: 3'ABL1 / 5'BCR EXAMPLE: der(22) EXAMPLE: 5%
EXAMPLE: t(8;21)(q22;q22) EXAMPLE: 5'RUNX1 / 3'RUNXT1 EXAMPLE: der(8) EXAMPLE: 5%

Individual Region Genomic Gain / Loss / LOH

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Chr # Gain / Loss / Amp / LOH Minimal Region Genomic Coordinates [Genome Build] Minimal Region Cytoband Diagnostic Significance (Yes, No or Unknown) Prognostic Significance (Yes, No or Unknown) Therapeutic Significance (Yes, No or Unknown) Notes
EXAMPLE:

7

EXAMPLE: Loss EXAMPLE:

chr7:1- 159,335,973 [hg38]

EXAMPLE:

chr7

Yes Yes No EXAMPLE:

Presence of monosomy 7 (or 7q deletion) is sufficient for a diagnosis of AML with MDS-related changes when there is ≥20% blasts and no prior therapy (add reference).  Monosomy 7/7q deletion is associated with a poor prognosis in AML (add reference).

EXAMPLE:

8

EXAMPLE: Gain EXAMPLE:

chr8:1-145,138,636 [hg38]

EXAMPLE:

chr8

No No No EXAMPLE:

Common recurrent secondary finding for t(8;21) (add reference).

Characteristic Chromosomal Patterns

Put your text here (EXAMPLE PATTERNS: hyperdiploid; gain of odd number chromosomes including typically chromosome 1, 3, 5, 7, 11, and 17; co-deletion of 1p and 19q; complex karyotypes without characteristic genetic findings; chromothripsis. Do not delete table.)

Chromosomal Pattern Diagnostic Significance (Yes, No or Unknown) Prognostic Significance (Yes, No or Unknown) Therapeutic Significance (Yes, No or Unknown) Notes
EXAMPLE:

Co-deletion of 1p and 18q

Yes No No EXAMPLE:

See chromosomal rearrangements table as this pattern is due to an unbalanced derivative translocation associated with oligodendroglioma (add reference).

editHAEM5 Conversion Notes
Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification

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.[3]

Gene Mutations (SNV / INDEL)

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Gene; Genetic Alteration Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other) Prevalence (COSMIC / TCGA / Other) Concomitant Mutations Mutually Exclusive Mutations Diagnostic Significance (Yes, No or Unknown) Prognostic Significance (Yes, No or Unknown) Therapeutic Significance (Yes, No or Unknown) Notes
EXAMPLE: TP53; Variable LOF mutations

EXAMPLE:

EGFR; Exon 20 mutations

EXAMPLE: BRAF; Activating mutations

EXAMPLE: TSG EXAMPLE: 20% (COSMIC)

EXAMPLE: 30% (add Reference)

EXAMPLE: IDH1 R123H EXAMPLE: EGFR amplification EXAMPLE:  Excludes hairy cell leukemia (HCL) (add reference).


Note: A more extensive list of mutations can be found in cBioportal (https://www.cbioportal.org/), COSMIC (https://cancer.sanger.ac.uk/cosmic), ICGC (https://dcc.icgc.org/) and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.


editHAEM5 Conversion Notes
Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification

Put your text here and/or fill in the tables

Gene Mutation Oncogene/Tumor Suppressor/Other Presumed Mechanism (LOF/GOF/Other; Driver/Passenger) Prevalence (COSMIC/TCGA/Other)
CDKN2A Copy number loss Tumor Suppressor
NFKBIA
TP53
BIRC3
CCND2
BRAF p.V600E 0-19%
TRAF3
TNFAIP3
RB1
CDK4/MDM2
PTEN
JAK2
SOCS3
BRCA1/BRCA2
KRAS
MYC

Other Mutations

Type Gene/Region/Other
Concomitant Mutations EXAMPLE: IDH1 R123H
Secondary Mutations EXAMPLE: Trisomy 7
Mutually Exclusive EXAMPLE: EGFR Amplification

Epigenomic Alterations

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Genes and Main Pathways Involved

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Gene; Genetic Alteration Pathway Pathophysiologic Outcome
EXAMPLE: BRAF and MAP2K1; Activating mutations EXAMPLE: MAPK signaling EXAMPLE: Increased cell growth and proliferation
EXAMPLE: CDKN2A; Inactivating mutations EXAMPLE: Cell cycle regulation EXAMPLE: Unregulated cell division
EXAMPLE:  KMT2C and ARID1A; Inactivating mutations EXAMPLE:  Histone modification, chromatin remodeling EXAMPLE:  Abnormal gene expression program
editHAEM5 Conversion Notes
Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification

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.

Genetic Diagnostic Testing Methods

The only current method of diagnosis is tissue biopsy with immunohistochemistry. FDCS must be differentiated from other mesenchymal dendritic cell neoplasms, histiocytic and dendritic cell neoplasms, and non-hematopoietic tumors such as carcinoma, sarcoma, or melanoma which may show histologic similarities.

Familial Forms

  • None known

Additional Information

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Links

HAEM5:EBV-positive inflammatory follicular dendritic cell sarcoma

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References

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  1. 1.0 1.1 Chan JKC, et al., (2017). Follicular dendritic cell sarcoma, 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, p129-171.
  2. 2.0 2.1 Alaggio, Rita; et al. (2022-07). "The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms". Leukemia. 36 (7): 1720–1748. doi:10.1038/s41375-022-01620-2. ISSN 1476-5551. PMC 9214472 Check |pmc= value (help). PMID 35732829 Check |pmid= value (help). Check date values in: |date= (help)
  3. 3.0 3.1 3.2 Facchetti, Fabio; et al. (2021-10). "Follicular dendritic cell sarcoma". Pathologica. 113 (5): 316–329. doi:10.32074/1591-951X-331. ISSN 1591-951X. PMC 8720404 Check |pmc= value (help). PMID 34837090 Check |pmid= value (help). Check date values in: |date= (help)

Notes

*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.

*The hierarchical tumour classification structure displayed on this page is reproduced from the WHO Classification of Tumours with permission from the copyright holder, ©International Agency for Research on Cancer.

*Citation of this Page: “Follicular dendritic cell sarcoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated 09/1/2024, https://ccga.io/index.php/HAEM5:Follicular_dendritic_cell_sarcoma.