Follicular Dendritic Cell Sarcoma (FDCS) is an extremely rare tumor type. Although experts predicted the existence of this type of tumor in 1978, it was not fully recognized as an independent tumor until 1986. of cancer. It accounts for only 0.4% of soft tissue sarcomas, but its potential for recurrence and metastasis is quite significant and it is considered a tumor of intermediate malignancy. The major obstacle to the diagnosis and treatment of FDCS is misdiagnosis, as it has similarities in presentation and similar markers to Hodgkin and non-Hodgkin lymphomas, making diagnosis extremely difficult.
"Most cases of FDCS develop in the lymph nodes, but approximately 30% of cases develop in the perilymphatic sites."
Recent advances in cancer biology have led to the development of more accurate diagnostic methods and chemotherapeutic drugs, resulting in improved diagnosis and treatment of FDCS.
Follicular dendritic cells are mainly present in the center of lymphoid follicles, play an important role in regulating follicular responses, and are responsible for presenting antigens to B cells. Symptoms of FDCS vary depending on where the disease occurs. The most common symptom is painless swelling of the lymph nodes, but this symptom itself is non-specific as many other illnesses, including colds, can cause it. Other symptoms include cough, sore throat, difficulty swallowing, weight loss and fatigue.
"According to literature reports in 2000, approximately 12% of FDC tumor cases are associated with EB virus, but the role of EB virus in the pathogenesis of FDCS tumors remains unclear."
Due to the variability in patient medical records, no clear cause has been directly associated with FDCS. However, some evidence suggests that previous exposure to EBV or a diagnosis of Castleman disease may increase the risk of developing FDCS.
The proliferation characteristics of FDCS cells are similar to those of many other tumors, making their diagnosis complicated. These cells are large and binucleated and form clusters with lymphocytes, which makes it difficult to analyze their structure during staining. Therefore, pathological diagnosis requires the use of multiple methods, including morphological, cytochemical and electron microscopic analysis.
Cellular abnormalities found in FDCS tumors have been used for diagnosis. Characteristically, FDCs have a microtubule network structure and increased internal Clusterin. The microtubule network structure has an important influence on cell division, while Clusterin helps to clean up cell waste and is related to cell apoptosis.
In the early days of FDCS, the medical community had no data on the effects of chemotherapy and radiotherapy, so doctors could only try existing chemotherapy drugs. Most initial cases are treated simply with surgical resection or radiation therapy. However, as the treatment of FDCS progressed, physicians began to use CHOP, a common chemotherapy regimen similar to that for lymphoma.
"The CHOP regimen contains cyclophosphamide, doxorubicin, Oncovin and steroids, which act on different cancer cells through different pathways."
Although some FDCS patients have reported temporary improvement after CHOP treatment, the results are not consistent and there is still a need for improvement.
Recent efforts have focused on using PEG-liposomes to load doxorubicin, an approach that effectively increases drug concentration in tumors and reduces side effects. The technology takes advantage of the angiogenesis and leaky vascular characteristics of cancer tumors.
The study and treatment of FDCS continues to be influenced by research on other cancers. Despite the lack of funding for this type of cancer, there is potential to pave the way for innovative treatments for FDCS.
"Every advance may be a blueprint that is out of reach, but it all aims to eventually find a path to effectively treat FDCS."
Although some progress has been made in the diagnosis and treatment of FDCS, we have not yet found a clear cause and treatment plan. With the in-depth study of this disease, will there be new breakthroughs in the future?