DSRCT

DSRCT is classified as a rare and aggressive soft tissue sarcoma and childhood cancer. It is a violent and rare tumor that mostly occurs as multiple masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis.

William's disease is in the following areas
*Lungs -microscopic
*Mediastina
*Liver-Bulk of the disease
*Spleen
*Retroperitoneal Lymph node
*Left Testes
*Multiple bone masses including spinal cord
At the time of diagnoses, william's largest tumor was the size of a grapefruit.


The term “childhood cancer” is not an indication of the average patient’s age but, instead the cells that are affected by the cancer. DSRCT cancer cells are related to the primitive cells associated with children. We are born with the cells and they remain in the body (depending on your own unique biology) up to the age of 40, and in some age of 60 years. So while DSRCT is considered a childhood cancer it is not in reference to a patient’s real age at diagnosis but, to the primitive cells associated with children. In the case of DSRCT it is the small round blue cells that are considered primitive; therefore it is far more likely for children to get this disease than it is for adults.

DSRCT is associated with a unique chromosomal translocation (t11;22)(p13:q12) resulting in a EWS/WTI transcript that is diagnostic of this tumor. This transcript codes for a protein that acts as a transcriptional activator that fails to suppress tumor growth.

Resources for DSRCT patients and families.

http://scratchpad.wikia.com/wiki/Category:DSRCt
Database of patients, treatments, treatment facitlities with experience, and medical journal articles.

http://health.groups.yahoo.com/group/DSRCT/
This group is for people afflicted with Desmoplastic Small Round Cell Tumor (DSRCT). This is a forum for patients to share treatment stories and tips with each other.

http://www.gifttocure.org/
The mission of GiftToCure is to support individuals with DSRCT, and Ewing’s Sarcoma, and their immediate families by providing information, as well as emotional and financial support;Raise public awareness; and Sponsor research toward more-effective treatment and, ultimately, a cure for DSRCT.

https://www.facebook.com/#!/groups/27473069578/
DSRCT support group on FaceBook


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