follicular dendritic cell sarcoma
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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
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 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
80 cases was documented until 2011 in English literature
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
Hepatobiliary Pancreat Dis Int, Vol 10, No 4 • August 15, 2011
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
Histopathology
proliferation of spindle to ovoid cells that form fascicles, storiform patterns, and whorls
Plump, eosinophilic, fibrillary cytoplasm with indistinct cell border
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
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 benefitOnkologie 2007;30:381–384
Prognosis
As a low grade sarcoma with high local recurrence rate but low metastasis risk Overall recurrence: 43% Overall metastasis: 24% Overall mortality: 17% 2-year recurrence free survival: 62.3% 5-year recurrence free survival: 27.4%
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
Cancer. 1997;79:294–313.
Virchows Arch. 2006;449(2):148–158.
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