Desmoplastic Small Round Cell Tumor
by Gerry Webber
visitors since February 2000
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Desmoplastic Small Round Cell Tumor
by Gerry Webber
visitors since February 2000
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In October of 1999 I was diagnosed with DSRCT. Since I had never heard of this type of malignancy, I immediately began searching the web for information and soon found how little there was out there. There are several web sites dedicated to DSRCT (see Links) that have been published by persons such as myself, which I found to be very helpful in my search for information. Therefore, I decided to create a site too in hopes that it will prove helpful to others.
Appreciation:
I would like to thank the doctors, nurses and all the medical staff
that have been so helpful in my treatment. Especially Dr. Luciana Coura my primary
care oncologist along with Dr. Jose Bines of The National Institute of Cancer (INCA) in
Rio de Janeiro, Dr. Kushner of Memorial Sloan-Kettering Cancer Center in New York for the
generous sharing of his experience and prompt answering of our questions, and Dr. Bruno
and Dr. Flavia, my attending residents at INCA.
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. There are distinctive
clinical and histological characteristics that differentiate DSRCT from other small cell
tumors. 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. However, cases have been reported
in all age groups of both males and females and with tumors present in other parts of the
body. It would be unrealistic to guess at statistics on prognosis and survival rates
at this time.
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.
Treatments:
Treatment of DSRCT varies from patient to patient, but the most
effective treatments have consisted of early resection of the tumor(s) when possible and
high dose multi-agent chemotherapy followed by a stem cell transplant. Many cases
include a mid-chemo surgery to remove any residual disease and make the subsequent chemo
treatments more effective.
There are two main protocols reported in the literature: the P6
Protocol developed by Dr. Kushner at Memorial Sloan-Kettering Cancer Center in New York
and the Eicess EVAIA protocol used mainly in Europe. Both protocols use similar
chemo drugs, use extremely high doses of chemo, and have a similar number of
courses. Most successfully treated cases report the use of these, or variations of
these protocols. It must be remembered though, that these protocols are not the only
options. Each individual case is different and must be researched independently to
determine the best approach for treatment.
The
P6 Protocol:
The P6 Protocol consists of seven courses of chemotherapy with surgery
after the third course (if necessary), followed by a stem cell transplant. The
courses are described below or see Abstract #68 on the Research Abstracts page for Dr.
Kushner's 1996 article. Maximum tolerable doses for each course are calculated by
the patients weight and age.
Courses
1,2,3 and 6
cyclophosphamide
doxorubicin
vincristine
(Cyclophosphamide is administered with
two 6 hour infusions on the first 2 days.
Doxorubicin and vincristine are administered
by a 72 hour continuous infusion)
Courses
4,5 and 7
infofamide
etoposide
(Both infofamide and etoposide are administered
by 1 hour infusions over 5 days)
Suplimentary
Drugs
mesna
(Mesna is administered by intravenous
injection
with the cyclophosphmide in courses 1,2,3
and 6,
and with each infusion of courses 4,5 and
7. It is
used to prevent bleeding in the bladder and kidneys)
filgrastima (G-CSF)
(G-CSF, a blood cell growth stimulant, is
administered by subcutaneous injection
for 10 days after the completion of each
course)
Additional
Drugs
Zofran (anti-nausea)
Sulfametoxazol +
Trimetoprima (anti-pneumonia)
Ranitidina or Zantac
(anti-ulcer)
After each course, blood counts are monitored closely and transfusions of whole blood and/or platelets are given if blood counts get too low. Subsequent courses are started after blood counts rise back to normal levels, usually 18 to 20 days after the completion of the infusions.
Stem Cell
Transplant
thiotepa
carboplatin
The stem cell transplant procedure consists of first harvesting stem cells from the patient, administering high doses of thiotepa and carboplatin, then re-introducing the harvested stem cells into the blood stream along with G-CSF.
The Eicess EVAIA Protocol:
The Eicess EVAIA Protocol has been used mainly in Europe and has
been commonly used to treat Ewings Sarcoma. This protocol consists of eight courses
each consisting of:
Courses
1 through 8
vincristine
doxorubicin
infosfamide
etoposide
Suplimentary
Drugs
mesna
(Mesna is administered by intravenous
injection)
filgrastima (G-CSF)
(G-CSF, a blood cell growth simulate, is
administered by subcutaneous
injection
for 6 days after the completion of each
course)
Stem
Cell Transplant
(I don't have
specific information on the drugs
used, but stem cell
transplants are not
unique to either
protocol)