DSRCT is usually described as an aggressive tumor that typically emerges as multiple masses in the abdomen involving the regional
lymph nodes and the lining of the abdomen and pelvis. Young males seem to have a higher incidence of the disease, but a significant
number of female patients as well as some older individuals appear in case histories. Pamela was an example: Female/Age 35.
The most common symptoms include abdominal pain, abdominal mass and symptoms of gastrointestinal obstruction.
Para testicular, ovarian, parotid gland, brain and thoracic primary tumors have also been described. The cell of origin for this tumor is unclear.
The most common sites of spread are the liver, lungs and bones.
In many of the cases I have read about, reoccurrence develops in or around the liver or Vena Cava. Again, in Pamela's case,
the tumor returned, not in the abdominal area of origination, but around the Vena Cava and on on her liver.
Of course, every person is unique, and these are just personal observations. However, knowing what I know now, we would
have monitored the thoracic area much more carefully and frequently during Pamela's remission.
Searching the medical literature, the earliest reference to Desmoplastic Small Round Cell Tumor that I could find was in 1989
by Dr's. J. Rosai and W. L. Gerald*. He apparently later wrote a more descriptive paper in 1991 while at Memorial Sloan-Kettering Cancer Center in New York.
He was kind enough to review the slides of Pamela's specimen and confirm the diagnosis for us during the initial diagnostic confusion in 1995.
Although DSRCT is "aggressive" in the sense of being difficult to eradicate, it sometimes progresses very slowly and frequently responds remarkably
well to initial, highly aggressive treatment protocols. The difficulty seems to be in removing the tumor completely, since a very high percentage
of cases relapse within two to four years . This was our experience with Pamela. Looking back on her symptoms, it seems highly likely that
disease was present as early as May or June of 1995 (perhaps earlier), although she was not diagnosed until November, 1995 when surgery was performed.
Even after the initial surgery, her tumor grew very slowly from November 1995 until February 1996 when additional treatment began.
In reviewing other cases posted on the Web, I have noted some attempts in recent years to apply hormonal therapy.
Specifically, Androgen blocking has been used on the theory that some tumors may be sensitive to testosterone or some other male hormone.
Pamela received Leupron late in her treatment. Ironically, this was an idea that we posited to several oncologists early in 1996 when we wondered
why so many patients were young males. The doctors at the time generally dismissed the idea, but it has since become more acceptable.
For a medical description and additional details see the following link:
Desmoplastic small cell tumor with divergent differentiation.
Pediatric Pathology 1989;9(2):177-83 (ISSN: 0277-0938)
Gerald WL; Rosai J
Department of Pathology, Yale University School of Medicine, New Haven, Connecticut 06510.