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

13. Thanks for Your
attention!!