Clear-cell sarcoma

Clear-cell sarcoma (CCS), also known as clear-cell sarcoma of the soft tissue or melanoma of soft parts, is a rare form of cancer that primarily affects young adults. It is characterized by its clear cells and its similarity to melanoma, a type of skin cancer.

History

Clear-cell sarcoma was first described in 1965 by Dr. Franz M. Enzinger, a pathologist who specialized in soft tissue tumors. He noted the tumor's resemblance to melanoma, but its occurrence in the deep soft tissues of the body, such as tendons and aponeuroses, distinguished it as a unique entity.

Characteristics

Clear-cell sarcoma tumors are typically slow-growing and can be present for months or even years before they are diagnosed. They are most commonly found in the extremities, particularly the feet, but can occur anywhere in the body. The tumors are often deep-seated and attached to tendons or aponeuroses.

Histologically, clear-cell sarcoma is characterized by nests of uniform, round cells with clear or slightly eosinophilic cytoplasm. The cells often contain melanin, a pigment that is also found in melanoma cells. This has led to the alternative name, "melanoma of soft parts".

Genetics

Clear-cell sarcoma is associated with a specific chromosomal translocation, t(12;22)(q13;q12), which results in the fusion of the EWSR1 gene on chromosome 22 with the ATF1 gene on chromosome 12. This fusion gene is believed to play a key role in the development of clear-cell sarcoma.

Treatment and prognosis

Treatment for clear-cell sarcoma typically involves surgical removal of the tumor, often followed by chemotherapy or radiation therapy. However, clear-cell sarcoma is known to be resistant to many forms of chemotherapy, making treatment challenging.

The prognosis for clear-cell sarcoma is generally poor, with a five-year survival rate of around 50%. The tumor often metastasizes to other parts of the body, particularly the lungs, lymph nodes, and bones.

See also

• Soft tissue sarcoma
• Melanoma
• EWSR1
• ATF1