Desmoplastic Small Round Cell Tumors (DSRCT) – Symptoms and Causes
Overview
Desmoplastic small round cell tumors (DSRCT) are rare cancers that usually start in the abdomen. These tumors most commonly develop on the peritoneum, which is the tissue lining the inside of the abdomen and pelvis.
DSRCT belongs to the soft tissue sarcoma category – a diverse group of cancers that originate in connective tissues throughout the body.
This aggressive cancer can spread quickly to nearby organs including the bladder, colon, and liver. While DSRCT can affect anyone, young males are most frequently diagnosed with this condition.
Signs and Symptoms
Desmoplastic small round cell tumors (DSRCT) may cause several symptoms. These rare tumors typically affect the abdomen and pelvis. Below are common signs that patients might experience:
- Abdominal pain or discomfort
- Swelling or a lump in the abdomen
- Feeling of fullness
- Weight loss without trying
- Nausea or vomiting
- Constipation or other bowel changes
- Fatigue or weakness
- Night sweats
The symptoms often develop slowly. Many patients don’t notice problems until the tumor grows large. Some people may feel a hard mass in their abdomen when the disease is more advanced.
When to Contact a Healthcare Provider
See a doctor right away if you notice:
- Persistent abdominal pain lasting more than a few days
- Unexplained weight loss
- A lump or swelling in your abdomen
- Ongoing digestive problems that don’t improve
- Severe fatigue that interferes with daily activities
Don’t delay seeking medical care if you experience these symptoms, especially if they persist or worsen over time. Your doctor can order tests to find the cause of your symptoms.
Causes
The exact causes of desmoplastic small round cell tumors (DSRCT) remain unclear. Research suggests these rare cancers involve a specific genetic change.
Almost all cases show a specific chromosome change. Parts of chromosomes 11 and 22 swap places, creating a fusion gene. Scientists call this gene the EWSR1-WT1 fusion gene. This gene leads to abnormal protein production that can trigger tumor growth.
Unlike some cancers, DSRCT doesn’t appear to be hereditary. The gene mutation typically happens after birth rather than being passed from parents to children. Scientists believe this change occurs randomly in cells, though the exact trigger remains unknown.
Risk Factors
DSRCT (desmoplastic small round cell tumor) has limited known risk factors. This rare cancer typically affects young people. It particularly affects males between ages 10 and 30. Studies show males develop these tumors about four times more often than females.
No clear environmental, lifestyle, or family history connections have been established for DSRCT.
