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
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'.