Follicular Dendritic Cell Sarcoma

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Primary Author(s)*

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

Cancer Category/Type

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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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[1][3]

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

Chromosomal Rearrangements (Gene Fusions)

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%

Characteristic Chromosomal Aberrations / Patterns

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]

Genomic Gain/Loss/LOH

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Chromosome Number Gain/Loss/Amp/LOH Region
EXAMPLE 8 EXAMPLE Gain EXAMPLE chr8:0-1000000
EXAMPLE 7 EXAMPLE Loss EXAMPLE chr7:0-1000000

Gene Mutations (SNV/INDEL)

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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

Epigenomics (Methylation)

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Genes and Main Pathways Involved

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.

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.

Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications)

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Familial Forms

  • None known

Other Information

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Links

Inflammatory Pseudotumor-Like Follicular/Fibroblastic 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)

EXAMPLE Book

  1. Arber DA, et al., (2017). Acute myeloid leukaemia with recurrent genetic abnormalities, 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.

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.