Follicular dendritic cell sarcoma is a rare cancer that arises from follicular dendritic cells in lymph nodes or extranodal sites. It is classified as a dendritic cell neoplasm. Most cases occur in cervical or axillary lymph nodes, but extranodal cases can arise in organs like the spleen, gastrointestinal tract, liver, or lungs. Complete surgical resection is the primary treatment, but recurrence rates are high without adjuvant therapy. Prognosis is related to factors like tumor size and location, with intra-abdominal and larger tumors associated with poorer outcomes.

1. FOLLICULAR
DENDRITIC CELL
SARCOMA
R4洪逸平/VS顏厥全大夫

2. WHO classification of histiocytic
and dendritic-cell neoplasms
 Macrophage/histiocytic neoplasm
 Histiocytic sarcoma
 Dendritic-cell neoplasms
 Langerhans cell histiocytosis
 Langerhans cell sarcoma
 Interdigitating dendritic cell sarcoma/tumor
 Follicular dendritic cell sarcoma/tumor
 Dendritic cell sarcoma, not otherwise specified

3. Dendritic cell Sarcoma
 A rare disease
 Divided into interdigitating/follicular dendritic
cell sarcoma
 IDCS is more invasive
 Most of dendritic cell sarcomas (DCS) arise in
lymph nodes, about 1/3 involve the extranodal
sites

4. Follicular Dendritic cell
Sarcoma
 Nodal FDCS mostly affected cervical and
axillary lymph nodes
 Extranodal FDCS mostly affected intra-
abdominal organs and involves a wide variety
of sites, including spleen, gastrointestinal tract,
liver, soft tissue, skin, lung, and breast
 Metastatic disease is common in lymph nodes,
lung, and liver

5. Epidemiology
 80 cases was documented until 2011 in
English literature Pancreat Dis Int，Vol 10，No 4 • August 15，2011
Hepatobiliary
 Onset age is various (9-82), mean age is
about 50
 Female: male: 1.2:1
 Presentation: Painless, slow-growing mass
 Lab: anemia and elevated ALP in liver FDC
 Tumor is enhanced in PET

6. Etiology
 FDC express CD21 which is EBV receptor,
however the correlation is not well
documented
 Hyaline-vascular Castleman disease may be a
predisposing factor to FDC
 p53 pathway may play a role
 Epidermal growth factor receptor(EGFR)
expression has been investigated

7. Gross Pathology
 Solid and tan, may be some hemorrhage or
necrosis

8. Histopathology
 proliferation of spindle to ovoid cells that form
fascicles, storiform patterns, and whorls
 Plump, eosinophilic, fibrillary cytoplasm with
indistinct cell border

9. CYTOLOGY by FNA

10. Immunohistochemistry
 (+): CD21, CD35, and CD23
 (-): CD1a, lysozyme, myeloperoxidase, CD34,
CD3, CD79a, CD30, HMB-45, and
cytokeratins
 (+/-)Vimentin, desmoplakin, HLA-DR, CD68,
and epithelial membrane antigen
 Mostly(-): S100
 Others(+):
Clusterin, fascin, podoplanin

11. Treatment
 Complete surgical resection is the therapy of
choice
 Adjuvant radiation or chemotherapy showed
indeterminate benefit
 The optimal combination treatment for FDC
sarcoma has yet to be defined 2007;30:381–384
Onkologie
 Imatinib may be benefit

12. Prognosis
 As a low grade sarcoma with high local recurrence rate
but low metastasis risk
 Overall recurrence: 43%
 Overall metastasis: 24%
 Overall mortality: 17% Cancer. 1997;79:294–313.
 2-year recurrence free survival: 62.3%
 5-year recurrence free survival: 27.4%
Virchows Arch. 2006;449(2):148–158
 Poor prognostic factor:
 intra-abdominal location,
 size ≧ 6 cm,
 mitotic count ≧ 5 per 10 high-power fields
 coagulative necrosis
 Significant nuclear pleomorphism
 lack of adjuvant therapy

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