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follicular dendritic cell sarcoma

Friday 24 October 2003

Definition: Follicular dendritic cell (FDC) sarcoma is a very rare neoplasm showing morphologic and phenotypic features of FDCs. It occurs primarily in lymph nodes but also in extranodal sites.

Localization

- lymph nodes
- mediastinum (11745075, 20305533)
- lungs (11486174)
- breast (rare) (10196389)

Microscopical synopsis

- patterns of growth: diffuse, myxoid and fascicular
- neoplastic cells large, polygonal, with a slightly eosinophilic cytoplasm and oval or convoluted nuclei
- neoplastic cells intermingled with small lymphocytes, plasma cells and a few bizarre multinucleated giant cells.
- spindled tumoral cells (+/-)

The tumor can contain a major proportion of immature T cells, which may be connected to autoimmune phenomenas. (20305533)

Variants

- myxoid variant
- inflammatory

  • inflammatory pseudotumor-like follicular dendritic cell sarcoma

Associations

- myasthenia gravis (20305533)

Immunochemistry

- S-100+
- CD21+
- EMA+ (epithelial membrane antigen)
- CD21+/- (complement receptor)
- CD35+/- 9complement receptor)
- CD68+/-
- cytokeratins+/-
- clusterin+

Electron microscopy

- numerous interdigitating cytoplasmic processes with intercellular junctions of the serrated
- immature desmosomal or undifferentiated types

Differential diagnosis

- inflammatory pseudotumour
- interdigitating dendritic cell sarcoma
- true histiocytic lymphoma
- malignant Langerhans cell histiocytosis
- anaplastic large-cell lymphoma
- melanoma
- sarcomas

References

- A follicular dendritic cell sarcoma of the mediastinum with immature T cells and association with myasthenia gravis. Hartert M, Ströbel P, Dahm M, Nix W, Marx A, Vahl CF. Am J Surg Pathol. 2010 May;34(5):742-5. PMID: 20305533

- Perez-Ordonez B, Rosai J. Follicular dendritic cell tumor: review of the entity. Semin Diagn Pathol. 1998 May;15(2):144-54. PMID: 9606805