Follicular Dendritic Cell Sarcoma

Report
財團法人台灣癌症臨床研究發展基金會
FOLLICULAR DENDRITIC
CELL SARCOMA
R4洪逸平/VS顏厥全大夫
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
Dendritic cell Sarcoma
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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
Follicular Dendritic cell Sarcoma
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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
Epidemiology
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80 cases was documented until 2011 in English
literature Hepatobiliary Pancreat Dis Int,Vol 10,No 4 • August 15,2011
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
Etiology
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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
Gross Pathology

Solid and tan, may be some hemorrhage or necrosis
Histopathology

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proliferation of spindle to ovoid cells that form
fascicles, storiform patterns, and whorls
Plump, eosinophilic, fibrillary cytoplasm with
indistinct cell border
CYTOLOGY by FNA
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

Treatment
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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
Imatinib may be benefit Onkologie 2007;30:381–384
Prognosis

As a low grade sarcoma with high local recurrence rate but low
metastasis risk
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Overall recurrence: 43%
Overall metastasis: 24%
Cancer. 1997;79:294–313.
Overall mortality: 17%
2-year recurrence free survival: 62.3%
5-year recurrence free survival: 27.4% Virchows Arch. 2006;449(2):148–158.
Poor prognostic factor:
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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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