DSRCT Summary

Desmoplastic small-round-cell tumor is classified as a soft tissue sarcoma. It is an aggressive and rare tumor that primarily occurs as masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Reported sites of metatastic spread include the liver, lungs, lymph nodes, brain, skull, and bones.

The tumor is considered a childhood cancer that predominantly strikes boys and young adults.

The disease rarely occurs in females, but when it does the tumors can be mistaken for ovarian cancer.

Symptoms:

There are few early warning signs that a patient has a DSRCT. Patients are often young and healthy as the tumors grow and spread uninhibited within the abdominal cavity. These are rare tumors and symptoms are often misdiagnosed by family physicians. The abdominal masses can grow to enormous size before being noticed by the patient. The tumors can be felt as hard, round masses by palpating the abdomen.

First symptoms of the disease often include abdominal distention, abdominal mass, abdominal or back pain, gastrointestinal obstruction, lack of appetite, ascites, anemia, and/or cachexia.

Other reported symptoms include unknown lumps, thyroid conditions, hormonal conditions, blood clotting, kidney or urological problems, testicle, breast, uterine, vaginal, or ovarian masses

Treatment: 

DSRCT is frequently misdiagnosed. Adult patients should always be referred to a sarcoma specialist. This is an aggressive, rare, fast spreading tumor and both pediatric and adult patients should be treated at a sarcoma center.

There is no standard protocol for the disease; however, recent journals and studies have reported that some patients respond to high dose (P6 Protocol) chemotherapy, maintenance chemotherapy, debulking operation, cytoreductive surgery, and radiation therapy. Other treatment options include: hematopoietic stem cell transplantation, intensity-modulated radiation Therapy, radiofrequency ablation, stereotactic body radiation therapy, intraperitoneal hyperthermic chemoperfusion, and clinical trials.

Prognosis:

The prognosis for DSRCT remains poor. The 5-year survival rate of DRSCT is only approximately 15%. Prognosis depends upon the stage of the cancer. Because the disease can be misdiagnosed or remain undetected, tumors frequently grow large within the abdomen and metastasized or seed to other parts of the body.

There is no known organ or area of origin. DSRCT can metastasize through lymph nodes or the blood stream. Sites of metastatis include the spleen, diaphragm, liver, large and small intestine, lungs, central nervous system, bones, uterus, bladder, genitals, abdominal cavity, and the brain.

A multi-modality approach of high dose chemotherapy, aggressive surgical resection, radiation, and stem cell rescue improves survival for some patients. Reports have indicated that patients will initially respond to first line chemotherapy and treatment but that relapse is common.

Some patients in remission or with inoperable tumor seem to benefit from long term low dose chemotherapy, turning DSRCT into a chronic disease.

Introduction

Patty R
Group Moderator for DSRCT Support Group
DSCRT@yahoogroups.com

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'.