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 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 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 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 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 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 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: intra-abdominal location, size ≧ 6 cm, mitotic count ≧ 5 per 10 high-power fields coagulative necrosis Significant nuclear pleomorphism lack of adjuvant therapy Thanks for Your attention!!