Follicular Dendritic Cell Sarcoma



Follicular Dendritic Cell Sarcoma


Cyril Fisher, MD, DSc, FRCPath









Hematoxylin & eosin shows sheets of ovoid tumor cells with admixed adherent lymphocytes and prominent blood vessels.






Hematoxylin & eosin shows neoplastic cells that have distinctive oval nuclei with speckled chromatin, small nucleoli, and prominent nuclear membrane image. Indistinct cell margins give syncytial look.


TERMINOLOGY


Abbreviations



  • Follicular dendritic cell sarcoma (FDCS)


Synonyms



  • Dendritic reticulum cell sarcoma


Definitions



  • Malignant tumor of follicular dendritic cells



    • Antigen-trapping cells of accessory immune system


    • Normally in germinal center of lymph node


ETIOLOGY/PATHOGENESIS


Varied Etiologies



  • Most cases arise de novo


  • 10-15% arise in hyaline-vascular-type Castleman disease



    • Antecedent dendritic cell hyperplasia and dysplasia


  • Inflammatory pseudotumor-like variant is associated with EBV


CLINICAL ISSUES


Epidemiology



  • Age



    • Young and middle-aged adults


  • Gender



    • Slight female predominance


Site



  • Majority involve lymph nodes



    • Neck, mediastinum


    • Spleen, tonsil


    • Liver


  • Inflammatory pseudotumor-like variant



    • Females, in liver or spleen


  • Over 1/3 arise in extranodal sites



    • GI tract: Stomach, colon


    • Mesentery, mesocolon, mediastinum


    • Head and neck: Pharynx, palate


    • Soft tissue sites: Neck, axilla, breast


Presentation



  • Painless mass

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Follicular Dendritic Cell Sarcoma

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