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?
Rare type of cancer that forms in the abdomen.

What age group is typically affected by this condition?
Typically affects young adults and adolescents.

Where does the tumor usually occur?
Abdomen, particularly in the peritoneal cavity.

What are the common symptoms?
Abdominal pain, swelling, and masses.

Is Desmoplastic small round cell tumor aggressive?
Yes, it is an aggressive type of cancer.

How is the diagnosis typically made?
Through biopsy and imaging tests such as CT scans.

What is the treatment for this condition?
Surgery, chemotherapy, and radiation therapy.

Is Desmoplastic small round cell tumor curable?
Cure is rare, but treatment can help manage symptoms.

What is the prognosis for patients with this condition?
Generally poor, with a 5-year survival rate of around 15-30%.

Are there any genetic factors associated with this condition?
Yes, it is associated with a translocation between chromosomes 11 and 22.