Desmoplastic Small Round Cell Tumor

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive type of cancer that typically affects young adults, particularly males. It is characterized by the presence of small, round cells surrounded by a dense fibrous stroma, which gives it a desmoplastic appearance.

Causes and Risk Factors

The exact cause of DSRCT is unknown, but it is believed to be related to genetic mutations. The tumor often arises in the abdominal cavity, particularly in the peritoneum, and can also occur in other locations such as the pleura, pelvis, and scrotum.

Symptoms

The symptoms of DSRCT can vary depending on the location and size of the tumor. Common symptoms include:

• Abdominal pain or swelling
• Weight loss
• Fatigue
• Nausea and vomiting
• Bowel obstruction

Diagnosis

DSRCT is typically diagnosed through a combination of imaging studies, such as CT scans or MRI, and biopsy. The tumor cells are usually small and round, with a characteristic desmoplastic reaction surrounding them.

Treatment

Treatment for DSRCT usually involves a multimodal approach, including:

• Surgery to remove the tumor and affected tissues
• Chemotherapy to kill cancer cells
• Radiation therapy to shrink the tumor

Prognosis

The prognosis for DSRCT is generally poor, with a five-year survival rate of around 15-30%. However, early diagnosis and aggressive treatment can improve outcomes.

Current Research

Researchers are currently studying the genetic and molecular mechanisms underlying DSRCT, as well as exploring new treatments such as targeted therapies and immunotherapies.

Frequently Asked Questions (FAQs)

What is Desmoplastic small round cell tumor?
A rare and aggressive type of cancer that typically affects young adults and adolescents.

Where does Desmoplastic small round cell tumor usually occur?
Typically occurs in the abdominal cavity, but can also occur in other areas such as the lungs, liver, and lymph nodes.

What are the symptoms of Desmoplastic small round cell tumor?
May include abdominal pain, weight loss, fatigue, and bowel obstruction.

How is Desmoplastic small round cell tumor diagnosed?
Typically diagnosed through a combination of imaging tests, such as CT scans and MRI, and biopsy.

What is the prognosis for Desmoplastic small round cell tumor?
Generally poor, with a 5-year survival rate of around 15-30%.

Is Desmoplastic small round cell tumor hereditary?
No clear evidence to suggest that it is hereditary, but may be associated with genetic mutations.

What are the treatment options for Desmoplastic small round cell tumor?
May include surgery, chemotherapy, and radiation therapy, often in combination.

Can Desmoplastic small round cell tumor be cured?
In some cases, with aggressive treatment, but cure is rare and often depends on the stage and location of the tumor.

Are there any clinical trials for Desmoplastic small round cell tumor?
Yes, ongoing research and clinical trials are investigating new treatments and therapies.

How common is Desmoplastic small round cell tumor?
Extremely rare, with fewer than 200 reported cases in the medical literature.