Desmoplastic Tumors

What are Desmoplastic Tumors?

Desmoplastic tumors are rare but they are very aggressive tumors. They have a soft tissue and are considered a type of high-grade malignant cancer that infiltrates the tissue and the organs in the surrounding area. It can affect adults or young children.

Tumors are usually found deep within the body tissues. Often they are found in the pelvic area and abdomen. They can also be found in the pancreas, lung, hand and scalp.

What are the Symptoms of Desmoplastic Tumors?

Symptoms of a desmoplastic tumor depend on the location of the growth. The abdominal region including the fat linings and intestinal region are the most common areas in which these tumors can be found.

Symptoms include

When the desmoplastic tumors are found in the abdominal region, they usually come with pain and cramps, obstruction of the gastrointestinal tract, and a feeling that there is something in the stomach. Other symptoms, depending on the location of the tumor, can include anemia, chronic sinusitis, hormonal changes, back pain, and scoliosis.

Desmoplastic Tumors Causes

Currently, the specific causes or risks factors for developing desmoplastic tumors are unknown in terms of the disease’s specificity. However, the disease has been tied to primitive cells existing during childhood, meaning it operates as a kind of pediatric cancer. In fact, these tumors are particularly rare with less than 200 cases ever being reported and 85 % of those cases afflicting Caucasian people. Researchers have indicated a sort of chimeric relationship between desmoplastic tumors (or DSRCT) and Ewing’s sarcoma and Wilms’ tumors. In some cases they cluster with neuroblastoma and non-Hodgkin’s lymphoma to form small cell tumors.

Physicians have found that young men and boys are four times more likely to develop DSRCT than young women and girls and that these tumors are usually diagnosed among males between the ages of 10 and 30. DSRCT is often associated with a unique chromosomal translocation which doctors use to diagnose these tumors. This transcription essentially fails to suppress DSRCT growth. According to research, no current organ of origin is known for DSRCT and the tumor can metastasize throughout the blood stream or lymph nodes. Sites of DSRCT metastasis include the diaphragm, bones, uterus, spleen, large and small intestine, liver, lungs, bladder, genitals, central nervous system, abdominal cavity, and the brain.

How are Desmoplastic Tumors Treated?

Desmoplastic tumors can be treated with radiation therapy, chemotherapy, or invasive procedures. Chemotherapy is usually the first thing that is attempted. Surgical removal of the tumor is also a popular treatment. Surgical removal can be challenging though, depending on the location of the tumor. If these two methods are not successful, radiation therapy is administered.

Symptoms of a desmoplastic tumor can also be treated. Embolism may be suggested as a way to provide temporary relief and also to minimize blood loss for a surgical procedure.

Desmoplastic Tumors Prevention

Because DSRCT is a very aggressive tumor that typically develops in boys and young adult males, screening can be targeted and is a good means of prevention and metastasis. However, no DSRCT specific screening methods exist, though clinical trials are underway to develop a process. Consequently, DSRCT can often be misdiagnosed by doctors. Luckily, research has found that tumor markers such as serum cancer antigen 125 can be very effective for early detection of DSRCT. Because diagnosis is actually the most effective means of prevention and early detection is key, several screenings can be required on soft tissue masses in young males. A clear understanding of DSRCT by clinicians can also have an impact on early detection and treatment.

Last Reviewed:
October 05, 2016
Last Updated:
December 13, 2017