Desmoplastic small round cell tumor, also known as DSRCT, is a very aggressive form of tumor that can be very life threatening.
This type of tumor is usually found in the lining of the abdomen, but is sometimes found in other parts of the body. It is known to spread to the lungs, lymph nodes, liver, spleen, chest wall, skull, spinal cord, small intestine, bladder, brain,diaphragm, ovaries, testicles, pelvis and bones.
DSRCT is found mostly in males who are adolescents or young adults. A process called translocation occurs with this form of tumor; this is when a portion of chromosome 11 is translocated with some of chromosome 22. Doctors use this occurence to diagnose DSRCT. The disease is classified as a soft tissue sarcoma.
Risk Factors and Causes
There aren’t any known risk factors associated with desmoplastic small round cell tumors. The disease is known to pop up from primitive cells during childhood. Chromosomal translocations chromosome 11 and chromosome 22 causes this disease. When this happens, the body is no longer able to suppress tumor growth.
Signs and Symptoms
There are some early signs you can watch out for to warn you of DSRCT. Many of the patients that develop the disease are young and healthy when the tumors begin to grow.
Since this is a very rare tumor, it is oftentimes misdiagnosed. Commonly, the tumor grows very large before the patient begins to recognize symptoms. The tumors feel like hard round masses; this can be felt by palpitating the abdomen.
The symptoms that usually appear first include:
- Abdominal Mass
- Abdominal Pain
- Back Pain
- Abdominal Distention
- Gastrointestinal Obstruction
- Loss of Appetite
- Ascites
- Cachexia
- Anemia.
- Other signs and symptoms may include:
- Thyroid conditions
- Hormonal Conditions
- Kidney Problems
- Urological Problems
- Blood Clots
- Masses in the Testes, Breast, Vagina, Ovaries and Uterus
Treatment
Since DSRCT is often misdiagnosed, it is a good idea to visit a sarcoma specialist. Both adults and children should be treated at a Sarcoma Center once diagnosed with DSRCT. Some of the treatments that are available for DSRCT include:
- Chemotherapy
- Radiation Therapy
- Radiofrequency Ablation
- Hematopoietic Stem Cell Transplantation
- Intraperitoneal Hyperthemic Chemoperfusion
Outlook
Less than 20 percent of those diagnosed with DSRCT live longer than two or three years after diagnosis. Prognosis of DSRCT all depends on the stage of the tumor and its grade. Since the tumor often goes undetected for long periods of time, they tend to develop into large growths within the abdomen. Furthermore, this disease tends to metastasize (spread) into other parts of the body.
Prevention
Since DSRCT doesn’t have any avoidable risk factors, it is impossible to prevent the onset of this disease. If your or a loved one experience any of the aforementioned symtpoms, see a doctor immediately.
