What is a desmoplastic small round cell tumor?It is a very rare, rapidly growing cancer that develops in the abdominal cavity and manifests itself as a palpable abdominal mass. Besides the abdomen, the most common sites for DSRCT development are the lymph nodes, abdomen, chest, skull, spinal cord, small intestine, bladder, testicles, ovaries, brain, and pelvis (hip bone). The most common sites for distant tumor metastasis are the lungs, liver, lymph nodes, brain, skull, and bones. It is more common in male children than in women. Women are more likely to be misdiagnosed with ovarian cancer.
Disease Etiology (Cause)There is no definite well-known cause and identifiable risk factors, however, a link has been found between the presence of a unique chromosomal abnormality and the development of desmoplastic small cell tumors. Therefore, there is a high probability that the cause is genetic and that it is childhood cancer.
Signs and symptomsThe patient may suffer from the following signs and symptoms. The initial symptoms of the tumor are;
- A young and healthy person with a rapidly growing, hard palpable( touchable/ felt able mass)
Signs and symptoms where the disease becomes advanced
- Pain in the abdomen or back
- Absolute constipation
- Loss of appetite
- Symptoms of anemia such as lethargy, shortness of breath (difficulty in breathing), and pale color of eyes and skin.
- Ascites ( accumulation of fluid in the abdominal cavity)
- Weight loss
DiagnosisThe following diagnostic tests and procedures can be used to make a diagnosis. These are;
- Complete blood count (CBC)
- Using other imaging techniques such as abdominal USG (ultrasonography), X-rays, computed tomography, MRI, and PET to determine the characteristics of tumors, their location, size, and the extent to the regional and distant parts of the body.
- Tumor biopsy for histopathological examination