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The mystery of the rare tumor FDCS: Why is it so difficult to diagnose
Follicular dendritic cell sarcoma (FDCS) is an extremely rare tumor. Although Lennert predicted the existence of dendritic cell tumors in 1978, they were not fully recognized as a separate cancer until 1986 when they were characterized by Monda et al. FDCS accounts for only 0.4% of soft tissue sarcomas but has significant recurrence and metastasis potential and is considered an intermediate-grade malignancy. The major obstacle to treating FDCS is misdiagnosis. The diagnosis of FDCS is particularly difficult because of its similarities in presentation and markers to Hodgkin and non-Hodgkin lymphomas. Recent advances in cancer biology have led to better diagnostic tests and chemotherapeutic drugs, making accurate diagnosis and treatment of FDCS more feasible.
Follicular dendritic cells are localized to the germinal centers of lymphoid follicles and play an integral role in regulating germinal center reactions and presenting antigens to B cells.
Symptoms and manifestations
Most cases of FDCS occur in the lymph nodes, but about 30% of cases develop outside the lymph nodes. A large retrospective review of 51 patients in 1998 did not find any definitive patterns with respect to age, sex, race, or clinical presentation. The median age of the patients was 41 years (range, 14 to 76 years). Most cases presented with cervical and axillary lymphadenopathy, while 17 patients had tumors in the external lymph nodes, including the liver, spleen, intestine, and pancreas. Part. Due to the wide range of patient histories, no definitive cause has been associated with FDCS. However, there is some evidence that patients with a history of exposure to EBV or a diagnosis of Castleman disease have a higher risk of developing FDCS.
Diagnosis
Dyeing technology
The proliferation characteristics of follicular dendritic cells can be seen in many neoplastic diseases, including follicular hyperplasia, follicular lymphoma, etc. Although FDCS was recognized as a separate disease in 1986, it is still difficult to diagnose. FDCS cells are large, contain two nuclei, and form clusters with lymphocytes, making them difficult to distinguish during staining.
Cell mutation
The abnormal characteristics of cells in FDCS tumors are exploited for diagnostic purposes. The characteristic microtubule network structure (MTRS) of FDCS and the increase of intracellular clusterin can reflect multiple characteristics of cancer, including proliferation signaling, growth activation and replicative immortality.
Although FDCS has a variety of treatment options, the diagnosis and treatment of this disease still relies on improved cancer therapies such as CHOP and new drug cross-combination therapies.
Treatment methods
CHOP therapy
When FDCS was first discovered, there was no information about the effectiveness of chemotherapy and radiotherapy for this type of cancer. Doctors can only try to use existing chemotherapy drugs. As clinical data of FDCS patients accumulated, doctors began to use a commonly used chemotherapy regimen for leukemia and non-Hodgkin's lymphoma - CHOP, which is cyclophosphamide, doxorubicin, Oncovin and prednisone (CHOP). These drugs exploit different pathways in cancer cells to achieve their therapeutic goals.
Emerging Therapies
In recent years, the treatment of FDCS has been continuously improved. For example, PEG-liposome-encapsulated doxorubicin can increase the duration of the drug in the circulation. This method has been widely used because it can significantly reduce side effects by targeting the tumor. Pay attention to.
Research Status
Despite the lack of funding and low priority for research on FDCS, all advances in understanding and treating FDCS have come from research findings on other cancers. Scientists are increasingly discovering biological commonalities between seemingly different cancers, offering the potential for new treatments.
As our understanding of FDCS deepens, will there be more specific treatments for this cancer in future diagnosis and treatment options?
