WHAT IS DSRCT?

  DSRCT is a malignancy first described in the literature by Dr. Rosai of Memorial Sloan-Kettering Cancer Center in New York in 1991.The malignancy most commonly occurs in young males of adolescent age and is characterized as a large intra-abdominal mass with no apparent origin first spreading to the peritoneal surfaces, then to the liver, lymph nodes and/or lungs.   Since this lesion is normally not attached to an organ, it usually grows to a very large size and spreads to other parts of the body before the person experiences any ill effects of the disease.  Typical symptoms include intestinal blockage, swelling and pain in the abdomen and/or fever.  Since DSRCT is so rare, probably less than 200 cases reported, statistics are unreliable.  It appears that approximately 3/4 of cases are male with an average age in the late teens or early twenties and that over 95% of the cases had tumors located in the abdomen/pelvic area.

  Early reports describe a dismal prognosis for DSRCT due to its aggressive behavior and general non-responsiveness to known treatments of similar malignancies.  However, in the past several years, there have been treatments developed that show promising results with many patients experiencing complete remissions sustained for longer than two years.