Desmoplastic small round cell tumor

Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive form of cancer that primarily affects males in their adolescence and early adulthood. It is characterized by small, round, blue cells in a desmoplastic stroma.

Overview

DSRCT belongs to a group of cancers known as soft tissue sarcoma. It typically originates in the abdomen, but can also occur in other parts of the body. The tumor is often widespread at the time of diagnosis, making treatment challenging.

Symptoms

The symptoms of DSRCT can vary depending on the location and size of the tumor. Common symptoms include abdominal pain, a palpable mass, and signs of intestinal obstruction. Other symptoms may include weight loss, fatigue, and malaise.

Diagnosis

Diagnosis of DSRCT involves a combination of imaging studies, such as CT scan or MRI, and tissue biopsy. The tumor cells are typically positive for desmin and WT1 on immunohistochemistry.

Treatment

Treatment for DSRCT often involves a combination of surgery, chemotherapy, and radiation therapy. Despite aggressive treatment, the prognosis for DSRCT is generally poor, with a 5-year survival rate of less than 20%.

Research

Research into new treatments for DSRCT is ongoing. Current areas of investigation include targeted therapies, immunotherapies, and novel chemotherapy agents.

See also

• Soft tissue sarcoma
• Cancer
• Chemotherapy
• Radiation therapy

References

Desmoplastic small round cell tumor Resources
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