What is a desmoplastic small round cell tumour?
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 tumour 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 tumours. Therefore, there is a high probability that the cause is genetic and that it is childhood cancer.
Signs and symptoms
The patient may suffer from the following signs and symptoms. The initial symptoms of the tumour 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
Diagnosis
The 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 tumours, their location, size, and the extent to the regional and distant parts of the body.
- Tumour biopsy for histopathological examination
Treatment
Treatment options include surgical removal of the tumour, chemotherapy, radiation therapy, and hematopoietic stem cell transplantation.
What Support can we Give for Desmoplastic Small Round Cell Tumour?
Desmoplastic Small Round Cell Tumour is a rare cancer, meaning it is not as well known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. So, we provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other. If you suffer from rare cancer such as Desmoplastic Small Round Cell Tumour we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!
You can help us with your donation:
Without a Ribbon is a charity that works hard to aid those who suffer from rare cancers. You can help our cause in a variety of ways:
