Follicular dendritic cell sarcoma (FDCS) is an extremely rare tumor. Although Lennert predicted the existence of FDC tumors as early as 1978, this tumor was not fully recognized as a separate cancer until 1986, when Monda et al. described its characteristics in detail. FDCS accounts for only 0.4% of soft tissue sarcomas, but has significant recurrence and metastasis potential and is considered a cancer of intermediate malignancy. The major obstacle in treating FDCS is the difficulty in diagnosis.
Due to the similarities in clinical manifestations and markers between FDCS and Hodgkin's lymphoma and non-Hodgkin's lymphoma, the diagnosis of FDCS is quite complicated.
Follicular dendritic cells are mainly located in the germinal centers of lymphoid follicles and play an important role in regulating germinal center reactions and presenting antigens to B cells. Most cases of FDCS occur in lymph nodes, but about 30% of cases occur in extra-lymphatic sites. A 1998 retrospective review of 51 patients with FDCS did not find clear patterns in age, sex, race, or clinical presentation.
Among these 51 patients, the median age was 41 years (range, 14 to 76 years), and most cases presented with cervical and axillary lymphadenopathy, with 17 cases occurring in extranodal sites such as the liver, spleen, intestine, and pancreas.
Although FDCS has been recognized as a separate disease since 1986, it remains challenging to diagnose. The characteristic large, binucleated shape of FDCS cells makes them difficult to distinguish from lymphocytes during staining. The best way to identify them is through immunostaining techniques using FDC markers such as CD21 and CD35.
TreatmentWith the discovery of FDCS, there is little precedent for the effects of chemotherapy and radiotherapy. Initial treatment was mostly complete surgical resection or radiation therapy, but it was later found that the treatment effects were inconsistent. So doctors began using the CHOP chemotherapy regimen that is used for other cancers to treat FDCS. CHOP therapy consists of cyclophosphamide, doxorubicin, oxadiazine, and steroids, but its efficacy in FDCS patients is still not guaranteed.
With the deepening understanding of cancer biology, new treatments for FDCs are being developed, especially the emergence of PEG liposomes.
The development of PEG liposomes has greatly improved the delivery efficiency of doxorubicin. This method utilizes the characteristics of tumor vascular permeability and neovascularization to allow PEG liposomes to accumulate naturally at the tumor site, reducing the dosage and side effects, and is expected to become a new option for FDCS treatment.
In recent years, new chemotherapy drugs such as TAXOTERE and Gemcitabine have also begun to be used in the treatment of FDCS. Their effects on cancer cells have been significantly improved, showing better efficacy.
Due to the rarity of FDCS, funding for research into it is relatively limited, and advances in its treatment rely heavily on research findings from other, more common cancers. Although previous chemotherapy regimens have achieved certain results in FDCS, with the emergence of new treatments, more specific therapies for FDCS may be developed in the future.
As our understanding of cancer biology improves, exploring new treatment options and regimens is undoubtedly an exciting future for people with FDCS.
The prospects for treating FDCS are becoming clearer, but as science and technology continue to advance, can we expect more targeted therapies to emerge that will significantly improve patients' survival rates?