Follicular Dendritic Cell Sarcoma

Follicular Dendritic Cell Sarcoma

Sa A. Wang, MD

Lymph node involved by follicular dendritic cell (FDC) sarcoma. The neoplastic cells have indistinct cell borders, a moderate amount of eosinophilic cytoplasm, and oval or elongated bland nuclei.

Papanicolaou-stained scrape preparation of FDC sarcoma. The neoplastic cells are large and oval or spindle-shaped, with inflammatory cells in the background.



  • Follicular dendritic cell (FDC) sarcoma


  • FDC tumor

  • Dendritic reticulum cell sarcoma


  • Neoplastic proliferation of follicular dendritic cells

    • Immunophenotype supports FDC lineage


Normal FDCs

  • Localized to B-cell areas in primary and secondary lymphoid follicles

    • Form a meshwork via cell to cell attachments and desmosomes

    • Do not migrate

  • Trap and present antigens to B cells that are involved in B-cell proliferation and differentiation

    • Store antigen on cell surface as immune complexes

  • Closely related to bone marrow stromal progenitors

    • Have features of myofibroblasts

Etiology of FDC Sarcoma

  • Unknown in most cases

  • Small subset of cases of FDC sarcoma are associated with Castleman disease (CD)

    • Hyaline-vascular variant

    • FDC “dysplasia” has been reported in hyaline-vascular CD

  • Inflammatory pseudotumor-like variant of FDC sarcoma

    • Consistently associated with Epstein-Barr virus (EBV)

    • EBV is present in monoclonal form



  • Incidence

    • Rare

  • Age

    • Adults; median age: 40-50 years

  • Gender

    • Overall, no gender preference

      • Inflammatory pseudotumor-like variant shows female predominance

  • Ethnicity

    • No known predisposition


  • Often presents as slow-growing, painless mass

    • Lymph nodes

      • Cervical lymphadenopathy is most common

      • Other lymph node groups: Axillary, mediastinal, mesenteric, and retroperitoneal may or may not present

    • Extranodal sites

      • Waldeyer ring is most common, such as tonsil, oral cavity

      • Gastrointestinal tract

      • Soft tissue, skin

      • Thyroid, breast, mediastinum

      • Liver and spleen

    • Inflammatory pseudotumor-like variant FDC sarcoma

      • Often arises in intraabdominal sites: Liver, spleen, and peripancreatic area

  • Systemic symptoms

    • Uncommon in most patients with FDC sarcoma

    • Systemic symptoms are common in patients with inflammatory pseudotumor-like variant

      • Weight loss and fever

    • Paraneoplastic pemphigus can occur rarely


  • Most patients are treated by complete surgical excision

    • With or without adjuvant radiotherapy or chemotherapy

      • Various chemotherapy regimens have been used with limited success

      • Adjuvant radiotherapy may prolong disease-free survival


  • Most cases behave like low- to intermediate-grade soft tissue sarcoma

    • Local recurrences occur in > 50% of patients

    • Metastases occur in ˜ 25% of patients

      • Lymph nodes, lung, liver

    • 10-20% of patients ultimately die of the disease after many years

  • Poor prognostic indicators

    • Large tumor size (> 6 cm)

    • Intraabdominal location

      • Often in liver, spleen, or peripancreatic or retroperitoneal lymph nodes

      • Inflammatory pseudotumor-like variant of FDC sarcoma is more indolent

    • High-grade histologic features


General Features

  • FDC sarcoma cannot be distinguished from other malignant processes by imaging

  • CT and MR

    • Mass lesion, expansile

    • ± invasion of surrounding structures

  • Positron emission tomography (PET) shows abnormal radiotracer uptake



  • Mean: 5 cm; range: 1-21 cm


Histologic Features

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Follicular Dendritic Cell Sarcoma

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