While the existence of FDC tumors was predicted by Lennert in 1978, the tumor wasn't fully recognized as its own cancer until 1986 after characterization by Monda et al.[1][2] It accounts for only 0.4% of soft tissue sarcomas, but has significant recurrent and metastatic potential and is considered an intermediate grade malignancy.

It is a newly characterized cancer, and because of its similarities in presentation and markers to lymphoma, both Hodgkin and Non-Hodgkin subtypes, diagnosis of FDCS can be difficult.

[4] With recent advancements in cancer biology better diagnostic assays and chemotherapeutic agents have been made to more accurately diagnose and treat FDCS.

[7] Of these fifty-one patients, no conclusive pattern was found in regard to age, sex, race or presentation.

Other symptoms include cough, sore throat, difficulty swallowing, weight loss and tiredness.

In cases that present in extranodal sites outside of the head and neck region, organ specific symptoms are observed.

FDC cells are large, contain two nuclei, and form clusters with lymphocytes making them difficult to distinguish in staining.

[7] Because of the debate and difficulty of staining, pathologic diagnosis often requires morphologic, cytochemical and electron microscope analysis as well.

[12][13] MTRS contribute to microtubule formation of many structures, including the mitotic spindle, during cell division.

This contributes to many of the hallmarks of cancer, including proliferative signaling, growth activation, and replicative immortality.

Clusterin can be stained to help distinguish FDCS and is involved in the many important cancer hallmarks, including resistance to cell death and evading growth suppressors.

[14] At the time of the follicular dendritic cell sarcoma discovery, information on the effect of chemotherapy and radiation on it was nonexistent.

[15] At high doses it is very cytotoxic; its metabolite phosphoromide adds an alkyl group to the N7 position on guanine resulting in arrested growth and cell death.

The mechanism is not fully understood; it is thought that it interferes with the VEGF growth factors produced in and around the tumor microenvironment.

[18] Therefore, doxorubicin has a dual role in cancer treatment: it inhibits cell survival (causes apoptosis), and decreases angiogenesis.

Taxotere is similar to Oncovin used in CHOP; it irreversibly binds beta tubulin halting formation of microtubules.