FOLLICULAR DENDRITIC CELL SARCOMA

R4洪逸平 /VS顏厥全大夫

財團法人台灣癌症臨床研究發展基金會

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

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 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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