I am very familiar with desmoplastic small round cell tumor. It is a soft tissue sarcoma and I know many patients and their families with this disease. Since it is mostly a childhood cancer the pediatric oncologists have treated a great many of these patients using a multi-modality approach of high dose chemotherapy of Vincristine, Doxirubicin, Cytoxan cycled every 21 days with Ifosomide and Etopside (or the P6 protocol used for Ewings sarcoma) . . if there is significant response than the patient is offered surgery or intra-abdominal hyperthermic chemoperfusion and cytoreductive surgery. If that is successful than other treatment options include abdominal radiation, stem cell transplant, radiofrequency ablation, other chemo treatments (irinotecan and temodar) and last . . Clinical Trials.
DSRCT is a tough disease, but there are survivors. Anyone seeking more information about the disease should visit the website DSRCT.com There are also several other informative sites and a support group.
On 2/21/2008
DrBill wrote:
Anybody being treated for Desmoplastic small round cell tumor.??
I understand it is rare disorder and nobody has any experience with it.
Sloan Kettring claims claims they have treated six cases with high dose Cytoxan, but no follow up after six months. They dont know whether those patients are alive and or dead. Interestingly some of the inexeprienced centers using this high dose therapy without ever used this high dose Cytoxan 4500 mg dose which is horendous dose. One group tried in Columbus, and as they described the patient became a pulp and died. So always ask questions. The physicians have the power to put in a way that we may feel like that is the only way to go. When it comes to DSCRT if you ask any Oncologist they will not forth right in admitting that they do not know about it and instead will say " yes I have treated" One has to be careful just not to take the face value. A good doctor with great personality and manners can talk you into doing these extraordinary treatments with yout so called consent which covers their butt to cover for anything.
Dr.Bill