Childhood Soft Tissue Sarcoma: Treatment Information


Treatment for soft tissue sarcoma in children depends on the location of the tumor and therefore varies from patient to patient.

Treatment can be as simple as surgically removing the tumor through a small incision to extensive surgery and aggressive chemotherapy, with or without radiation.  The course of therapy is generally determined by the tumor type or grade (a pathological evaluation that shows how fast the tumor is growing) and the extent of disease (size and stage).  Treatment can be somewhat less aggressive for tumors that are small, lower grade, and completely removed by surgery.  

More aggressive therapy with chemotherapy and/or radiation is usually reserved for high-grade sarcomas that are larger and/or have spread to other parts of the body.

Desmoid Tumors

Tumors generally occurring in the arms or legs, but can arise anywhere.  Children with Gardner’s syndrome and familial polyposis coli are at higher risk of developing desmoid tumors.

Symptoms: Often present as a painless, slow growing mass.

Treatment: Surgery is the primary treatment for desmoid tumors.  If the tumor cannot be completely removed or the tumor recurs, other treatment can be considered.  radiation may be used to limit local recurrence.

Outcome: The chance for recovery is excellent with the complete removal of the tumor.  Factors that increase the risk of recurrence include diagnosis at a young age, aggressive features on pathology, incomplete removal of the tumor, and underlying Gardner’s syndrome or familial polyposis coli.  It is recommended that children who have this type of tumor be evaluated for a specific mutation (change) in the APC gene.  Some children with desmoid tumors may need to be screened for colon cancer

Desmoplastic Small Round Cell Tumor (DSRCT)

Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive tumor.  This cancer almost always arises in the abdomen, but can develop in other parts of the body.  This cancer spreads locally to the lymph nodes and the lining of the abdomen.  It can also spread to the lungs, liver and bones.

Symptoms: Symptoms depend on the primary site and extent of disease when diagnosed.  Patients with abdominal tumors often present with an enlarged abdomen which is painful and sometimes with vomiting and weight loss.

Treatment: Desmoplastic small round cell tumors are often treated with chemotherapy, radiation and surgery.  It is important to surgically remove all of the disease to eliminate the cancer.  chemotherapy is sometimes used to shrink tumors prior to surgery.

Outcome: The overall prognosis (chance of recovery) for patients with DSRCT is poor - less than 20% of patients are expected to live more than 5 years after diagnosis.  The chance for survival is higher if doctors are able to surgically remove all tumors.

Malignant Peripheral Nerve Sheath Tumor (MPNST)

These tumors tend to arise along nerves and usually affect the extremities arms or legs.  They can also arise in the abdomen, head and neck region.  This cancer can be associated with a condition called neurofibromatosis.  Your doctor will determine if your child has this condition.

Symptoms: Symptoms depend on the primary site of disease, but the cancer often presents as an enlarging mass with pain or changes in sensation.

Treatment: Treatment depends on the size and the grade (a pathological evaluation that shows how fast the tumor is growing).  Small and low-grade (less aggressive) tumors can usually be treated with just surgery.  Larger and high-grade (more aggressive) tumors may require treatment with radiation and chemotherapy in addition to surgery.

Outcome: The overall prognosis (chance of recovery) for patients with small, low-grade MPNST is excellent.  The prognosis for patients with large, high-grade MPNST is still generally good, but these patients require additional therapy with radiation and/or chemotherapy.  

Rhabdoid Tumors

This is a rare, but very aggressive form of cancer that tends to affect infants.  The two most common primary sites are the kidney and brain.  An aggressive treatment approach is often used, but most rhabdoid tumors spread to other parts of the body despite these efforts.

Synovial Sarcoma

This tumor usually develops near a joint in the leg, but can occur in other places in the body, including the lungs.  Within the tumor, a specific genetic abnormality is often identified where part of one chromosome is attached to another.

Symptoms: Symptoms often are an enlarging, painless mass, usually near the knee.

Treatment: Most synovial sarcomas are treated with a combination of surgery, chemotherapy and radiation.  It is important to try to completely remove the tumor.  The addition of radiation and chemotherapy to treatment protocols has improved survival in patients with synovial sarcoma.

Outcome: With a combination of surgery, chemotherapy and radiation, more then 70% of patients are expected to be long-term survivors.  

Research Studies

The majority of children with cancer participate in research studies.  This high rate of participation has been essential to improving the cure rates for children’s cancer.

Researchers design various studies to improve treatment and advance the understanding of cancer and its causes.  Clinical trials are carefully reviewed and must be approved through a formal scientific process before anyone can be enrolled.  If there is a research study “open” that your child is “eligible for,” you may be asked to allow your child to participate.  It is also possible that your child will be asked to participate in more than one study.

Whether an individual is eligible for a particular study may depend on age, location of the cancer, the extent of the disease and other information.  Researchers usually must limit their study to some of these characteristics to have a scientifically valid study.  Further, researchers must follow exactly the same restrictions throughout the study. 

If your child is eligible to participate in one or more study, your doctor will discuss these with you during an initial treatment conference (also called informed consent conference).  The doctor will describe the study, potential risks of participation, and other information you need to decide whether or not you would like your child to participate in the study.  You always have the choice to participate or not in research studies.

If you do choose to have your child participate in a study, your doctor will explain what type of information you will receive about the results of the study.  The overall results of the research study will be published to inform the public and other researchers.  No study will publish any information that identifies an individual. 

Visit the Clinical Trials section of this website to learn more about the various kinds of research studies.

Updated July, 2011

 

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