The prognosis for DSRCT is very poor and has a 5 year survival rate of around 15 percent although some people who have DSRCT with localized tumors that are outside of the abdomen can respond better to the treatment.
Although DSRCT often comes back even after the initial treatment that resulted in success.
The median overall survival of DSRCT is between 28 months to 60 months.
The survival rate of DSRCT is a 5 year survival rate of 15 percent to 25 percent.
Although some analysis has also shown there have been higher survival rates and most particularly when the tumors in DSRCT are treated before they have spread to other parts of the body.
DSRCT is not hereditary although a genetic abnormality is involved in DSRCT although DSRCT is acquired during the person's lifetime and not inherited by a parent.
The specific genetic change in DSRCT involves a translocation between chromosomes 11 and chromosomes 22 which leads to the fusion of the WT1 genes and EWS genes but the genetic alteration is not passed down through families.
The way you test for desmoplastic small round cell tumors or DSRCT cancer is through a combination of tests which includes imaging tests, biopsies and also molecular analysis.
A biopsy which is often a needle biopsy or even a surgical biopsy is crucial to obtain a tumor sample for microscopic examination.
Also imaging tests such as CT scans, PET scans and MRI scans can also help to determine the size of the tumor as well as the location and whether or not it has spread.
A molecular analysis which includes FISH or fluorescence in situ hybridization and RT-PCR helps to identify the specific genetic markers like the EWSR1-WT1 translocation that is also characteristic of DSRCT cancer.
The age that people get desmoplastic small round cell tumors is often between the ages of 15 to 30 years of age.
Although anyone can develop or get DSRCT cancer it is rare in older adults and children and the median age of DSRCT cancer or desmoplastic small round cell tumors at diagnosis is often reported as 19 years to 23 years of age.
The majority of cases of desmoplastic small round cell tumors or DSRCT are diagnosed in young males between that ages of 10 to 30 years of age.
The incidence of DSRCT is very low and has an estimated incidence of 0.2 to 0.3 cases per million people.
It's also been shown through studies that the incidence of DSRCT is higher in males and peaks in adolescents and young adults and most particularly in people between 20 to 30 years of age.
It's estimated that 200 or fewer people have had or have DSRCT.
Less than 200 cases of DSRCT also known as Desmoplastic Small Round cell Tumors have been reported since DSRCT cancer has been first described in the year of of 1989.
DSRCT is a very rare type of cancer and mainly affects young males between the ages of 10 to 30 years of age.
DSRCT is the rarest cancer and is a very aggressive type of sarcoma cancer which mainly affects young adults and children typically in their abdomen.
DSRCT is estimated to only affect around 20 young people in the United States each year and fewer than 200 cases of DSRCT cancer have been reported since it was first identified in 1989.
DSRCT can be cured in a small percentage of cases although complete cures of DSRCT cancer is rare.
When feasible complete surgical resection, has been shown to improve survival and can potentially lead to a cure of DSRCT in some cases.
Although because DSRCT is an aggressive form of cancer many people do experience a relapse even with treatment.
Tumors with DSRCT that are outside the abdomen and those that have not spread to other organs often respond better to treatment and DSRCT that has spread to other parts of the body also known as metastatic are more difficult to cure.
Complete surgical removal of the DSRCT tumor is a key factor in improving a persons outcomes and potentially achieving a cure of DSRCT.
The exact causes of DSRCT cancer is not known although it's believed that DSRCT cancer is a genetic disorder that involves a chromosomal translocation between the chromosomes 11 and chromosomes 22 which result in the fusion of the WT1 genes and EWSR1 genes.
This fusion then leads to the production of an abnormal protein which disrupts growth and differentiation, which contributes to the development of DSRCT cancer.
Other potential factors that can put someone at risk for DSRCT cancer include male gender, young age, certain genetic mutations and even exposure to environmental toxins although it's not well established.
DSRCT cancer is also known as Desmoplastic small round cell tumor cancer and is a rare and aggressive type of soft tissue sarcoma.
DSRCT cancer or desmoplastic small round cell tumor cancer is often found in the abdomen of young males and it's characterized by the presence of small, round cancer cells that are embedded in a dense and scar like tissue.
DSRCT cancer is a rare cancer and there's only a few hundred cases of DSRCT cancer reported worldwide.
DSRCT also mainly affects young adults and adolescents often between the ages of 10 years old and 30 years old and it often originates in the abdomen and most particularly within the lining of the abdomen and pelvis.
DSRCT cancer is also known for it's aggressive nature as well as tendency to spread to other parts of the body, which includes the lymph nodes, liver and the lungs.
A hallmark of DSRCT is also the presence of a specific genetic abnormality that is called the EWS-WT1 fusion gene.
The prognosis for DSRCT cancer remains poor even with aggressive treatment approaches, and has a 5 year survival rate of around 15 percent.
The symptoms of DSRCT cancer include weight loss, nausea and vomiting, changes in bowel habits such as diarrhea and constipation, abdominal swelling or mass and abdominal pain.
DSRCT cancer is often diagnosed through imaging scans such as PET scans, MRI scans and CT scans that are used to visualize the tumor and the extent of the tumor.
Treatment for DSRCT cancer include maintenance therapy, Hyperthermic Intraperitoneal chemotherapy, radiation therapy, chemotherapy and surgery.
Surgery to remove the tumor in DSRCT cancer is a crucial part of treatment, which aims to remove as much of the tumor as possible.
Chemotherapy is often used to shrink the DSRCT tumor before surgery and to also target the cancer cells which may have spread.
Radiation therapy may also be used to target and destroy any cancer cells, most particularly in cases where surgery is not feasible or when the tumor has spread.
And Hyperthermic intraperitoneal chemotherapy is a procedure that involves delivering of heated chemotherapy drugs directly into your abdominal cavity after surgical removal of the tumor.
And maintenance therapy can involve chemotherapy or targeted therapies to help prevent recurrence.