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Clear cell sarcoma is deemed highly aggressive due to its early tendency to metastasize, genetic mutations, and difficulty in early diagnosis and effective treatment
Clear Cell Sarcoma CCS tends to have a mild clinical presentation at presentation, which results in delayed diagnosis.
Clear cell sarcoma (CCS), or malignant melanoma of soft parts, is a rare, extremely aggressive soft tissue sarcoma, which accounts for less than 1% of soft tissue sarcomas. Though extremely rare, CCS is infamous for its aggressiveness, aggressive metastatic potential and poor prognosis. Dr Chetana Bakshi, Consultant Medical Oncologist, Jupiter Hospital Thane shares all you need to know:
One of the most important reasons for its virulence is its ability to spread quickly to distant locations, particularly the lungs and bones. CCS tends to have a mild clinical presentation at presentation, which results in delayed diagnosis. By the time symptoms are noted and the tumor is diagnosed, it is often already advanced with metastasis. This quick dissemination to distant organs is one of the main reasons for its poor prognosis.
The other reason for the aggressiveness of CCS is its genetic and molecular profile. Clear cell sarcomas characteristically contain a chromosomal translocation, most frequently t(12;22) (q13;q12), which creates the fusion between the EWSR1 gene and ATF1 gene. This variant is linked with the production of a fusion protein that causes abrogation of cellular processes, subsequently causing tumorigenesis. The molecular features of CCS also render it resistant to the majority of conventional chemotherapy regimens, making management even more difficult.
Histologically, CCS shows clear cells in a characteristic nests and cords arrangement in the tissue. The transparent nature of the tumor cells owing to high content of glycogen is responsible for the “clear cell” description. The tumor cells tend to have a lot of cellular pleomorphism, which is evidence of aggressive tumor growth and early invasion into nearby tissues. The tumor’s invasive potential into muscle, bone, and skin increases its metastatic potential and complicates surgical resection.
Clear cell sarcoma’s diagnostic difficulty adds to its aggressive behavior. Its appearance may mimic other, more prevalent tumors, such as malignant melanoma and other sarcomas, which may result in misdiagnosis and delayed treatment. Consequently, proper treatment modalities are frequently not initiated early enough to significantly alter outcomes.
Although it is rare, CCS has been a focus of research because it is aggressive. The treatment is usually surgery combined with radiation and chemotherapy, though chemotherapy resistance by the tumor usually precludes the use of these treatments. More recent therapies that are targeted toward correcting the genetic mutations causing CCS are being tested, but they are still in experimental phases.
In summary, clear cell sarcoma is deemed highly aggressive due to its early tendency to metastasize, genetic mutations, and difficulty in early diagnosis and effective treatment. Though its rarity does not make it as popularly known, its rapid tendency to spread and its resistance to conventional therapies make it a serious challenge in clinical environments.
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