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Follicular dendritic cell sarcoma

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  • 1. FOLLICULARDENDRITIC CELLSARCOMAR4洪逸平/VS顏厥全大夫
  • 2. WHO classification of histiocyticand 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 cellSarcoma 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 Yourattention!!