Is desmoplastic cancer curable?

Conclusions. DSRCT is a rare but often fatal disease that mainly affects younger male patients. Those with intra-abdominal DSRCT have a poorer prognosis, although surgical resection for localised disease and radiotherapy in the metastatic setting are associated with improved survival.

How many cases of DSRCT are there?

DSRCT is extremely rare. Fewer than 200 cases have been reported, about 85 percent of them in Caucasian people. Boys and young men are about four times as likely to have DSRCT as girls and young women. It is usually diagnosed in males between ages 10 and 30.

What is the survival rate of DSRCT?

The prognosis of DSRCT patients is very poor, overall survival is approximately 30% to 55% despite chemotherapy, radiotherapy, and aggressive surgical resection.

What is a desmoplastic small round cell tumor?

Desmoplastic small round cell tumors, or DSRCT, are tumors that grow in the abdomen and pelvic area of the body. Desmoplastic small round cell tumors are a type of soft tissue sarcoma, which is a type of cancer that forms in the connective tissue of the body.

What is Desmoplastic?

Listen to pronunciation. (DES-moh-PLAS-tik) Causing or forming adhesions or fibrous connective tissue within a tumor.

Is DSRCT hereditary?

The genetic change involved in DSRCT is acquired throughout a person’s lifetime and is not inherited .

What are the symptoms of DSRCT?

Symptoms of DSRCT can include abdominal pain, cramps, abdominal distention, a feeling of fullness and constipation. Patients often present with an abdominal mass. Patients typically receive imaging studies, including computed tomography (CT) and magnetic resonance imaging (MRI).

What is a round cell tumor?

The term round cell tumor describes a group of highly aggressive malignant tumors composed of relatively small and monotonous undifferentiated cells with increased nuclear-cytoplasmic ratio.[1] Malignant small round cell tumors (MSRCT) is a term used for tumors composed of malignant round cells that are slightly larger …

What is a desmoplastic stromal response?

Desmoplasia is the result of increased synthesis of extracellular matrix proteins and collagen by stromal cells. It is considered to be a reaction and response of the host tissue against invasive cancer cells.[5]

What does desmoplastic mean?

What is desmoplastic small round cell tumor?

Summary Summary. Desmoplastic small round cell tumor (DSRCT) is a rare type of soft tissue cancer (sarcoma) that usually begins in the abdomen. It primarily affects children and young adults and is more common in males.

What is a small round cell tumor called?

Also called: DSRCT, polyphenotypic small round cell tumor. Desmoplastic small round cell tumor (DSRCT) is a type of cancer. It is usually found in the abdomen (belly), but it can also occur in other parts of the body. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue.

What is a DSRCT tumor?

Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue. The tumor has a specific genetic abnormality that helps confirm the diagnosis. DSRCT in the abdomen may not be found until the tumors have grown large. As a result, often the disease has spread to the liver, lymph nodes, lungs or bones by the time it is diagnosed.

What is the survival rate for patients with DSRCT?

Because so few people have DSRCT, only a small amount of information on treatment results is available. Currently DSRCT has a 15 percent five-year survival rate. However, researchers are looking for treatments that will improve those odds.