Thursday, March 24, 2011


Patty R
Group Moderator for DSRCT Support Group
DSRCT (desmoplastic small round cell tumor) is considered a childhood cancer
(sarcoma) - not because of the age of the patient but because of the TYPE of
call that is affected. DSRCT is a cancer of the 'primitive cells' (one of the
ten types of blue celled cancers) most often associated with childhood. Because
of this association DSRCT is mostly a cancer that affects children, adolescents
and young adults and so it is the PEDIATRIC oncology most familiar with the
disease. Many times adults with DSRCT between the ages of 20 to 50 years of age
go to an adult oncologist who has never treated or even seen or heard of
desmoplastic small round cell tumor. I cannot emphasize enough how important it
is to find an oncologist who is experienced with DSRCT or at least specializes
in SARCOMA. Keep in mind that in terms of disease 20 percent of all sarcoma
cases are in the pediatric, adolescent and young adult age group . . while only
1 (ONE) percent of all sarcoma is found in the adult cancer population. An adult
oncologist can go there entire career never seeing a sarcoma. Your nephew should
be seen by someone who is either a pediatric oncologist or a sarcoma specialist
(Sarcoma Center).

We have seen far too many grim prognosis and incorrect treatments proposed by
adult oncologists who have no idea at all how to treat DSRCT - and this is a
cancer that leaves very little leeway for delayed treatment or to be
underestimated. It is an aggressive cancer best treated by complete surgical
removal. Because the disease is aggressive and often already at stage IV at
diagnosis than high dose chemotherapy (P6 protocol) is often given prior to any
surgical intervention. Chemo is given first for several good reasons . .
primarily to shrink and/or the disease as much as possible before surgery and to
also establish whether that patients unique cancer will respond to chemo or not.
In general, depending on the tumor burden (which can be in the hundreds of small
and midsize tumors) a patient will require between one and two surgeries. Once
the patient is 'no evidence of disease' . .the treatment becomes far trickier
because it is maintaining the disease 'free' status that is the most difficult
part of DSRCT.

Many through surgery can reach 'no evidence of disease' and unfortunately
oncologists unfamiliar with DSRCT often send their patients out the door and
tell them they are 'cured'. We have found through this group that this is not
the case . . DSRCT can return aggressively at some undetermined point in time.
Thus DSRCT requires a longer treatment time period than most other types of
cancer . . so chemotherapy should continue with possible further treatment
(depending on situation) of radiation and/or stem cell transplant. Some patients
are electing to go with surgery followed by a 'heated chemo' bath directly into
the tumor bed (this is being done at MD Anderson by Dr Hayes-Jordan - who now
has extensive experience treating DSRCT - but other surgical oncologists also
offer this treatment).

Even once traditional treatment is finished and there is 'no evidence of
disease' there is no guarantee that the cancer will not return at an
undetermined amount of time. Because of this uncertainty a patient should be
'monitored' for at least five years after treatment 'ends'.


  1. Wendy, I'm with you on the frustration. I feel so helpless to hear my kid say that even HE is frustrated & tired & just wants to go home, added to the other feelings he has related to the treatment he is going thru.
    It's frustrating to know that he knows chemo & radation is not a 100% guarantee that he will be cancer free.
    My 15 year old son Zeph is doing his last day of chemo on the P6 protocol at MSKCC's not the end however as he is still facing his second tumor resection on April 28th and I-8H9 radiation trial May or June.
    Love and prayers to you and yours for the strength to continue to fight, as you may very well know, we all always need to reach deep down to find it,
    Irma Sanchez
    Zeph's mom
    dx 8/2010

  2. Our methodology of treating DSRCT is seriously broken! It will take a little while to explain, so please bear with me.

    Here is one thing that is absolutely certain: People who take the diabetes drug called Metformin have a 40% better chance of not getting cancer. We have known this for almost six years. See:

    Metformin and reduced risk of cancer in diabetic patients April, 2005

    Metformin and Cancer Aug, 2009

    Diabetes drug can reduce risk of cancer, researchers find Sept, 2010

    So, since I do not want to get cancer, I went to my doctor to get a prescription for Metformin. He refused to give me a prescription for Metformin since Metformin is not "approved" for cancer. WHAT?? Metformin is one of the most harmless drugs known to man. Why isn't it approved? My doctor is a very good doctor and he was very patient with me - he explained "the facts of life" or, in this case, "the facts of death." Metformin is now a generic drug which means that any company can make it and those who were going to get very rich from Metformin now have their money and no one else can make billions of dollars from it. A company can only make billions of dollars when it is the only company making the drug. A drug gets "approved" by some company doing clinical trials for that drug. Clinical trials cost many millions of dollars and are only done by a company with the expectation that they will get their millions back plus much more. So, it should now be clear why Metformin is not approved for cancer. Since DSRCT is such a rare cancer, no company is going to make much money by doing clinical trials for it. The fact is, they would probably lose money.