Choldhood Soft Tissue Sarcomas

Soft tissue sarcomas arise from tissues such as fat, muscle and nerves. Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood. Others include: synovial sarcoma, malignant fibrosis histiocytoma, fibrosarcoma and others.

Rhabdomyosarcoma

Rhabdomyosarcoma originates in a striated muscle anywhere in the body. Rhabdomyosarcoma is the most common type of sarcoma found in the soft tissues of children.

Risk Factors
The known risk factors for Rhabdomyosarcoma are:

• Congenital anomalies
• Genetic disorders such as Li-Fraumeni syndrome and neurofibromatosis.

Symptoms
Most patients present with a mass in a body region without ahistory of trauma to that area. Also, there may be a disturbance of a normal body function by an enlarging tumor. The presenting features depend on the site from which the tumor arises.

Diagnosis
If your child has symptoms, the doctor may do x-rays and blood tests. If it is suspected that your child may have Rhabdomyosarcoma, your doctor may do a biopsy. A biopsy is removing a piece of tissue from the affected area. This tissue will be examined under a microscope to see if cancer cells are present.

Prevention
There is currently no way to prevent Rhabdomyosarcoma.

Staging
Once rhabdomyosarcoma is found, more tests will be done to find out if the cancer cells have spread to other parts of the body. There are several staging systems for rhabdomyosarcoma. The treatment options are based on size, location, and how far and where the cancer has spread.

Stage 1
Cancer is found in the eye, head and/or neck, or near your sex organs and bladder.

Stage 2
Cancer is located in only one area (but in none of the areas in Stage 1), is smaller than 2 inches across (5 cm) in size, and has not spread to the lymph nodes.

Stage 3
Cancer is located in one area (but not one of the areas in Stage 1), is greater than 2 inches across (5 cm) in size, and may have spread to the lymph nodes found nearby the cancer.

Stage 4
Cancer has spread and is found in more than one place at the time of diagnosis.

Recurrent
Recurrent disease means that the cancer has come back after it has been treated. It may be back in the area where it started or in another part of the body.

Treatment
The treatments that are used to treat Rhabdomyosarcoma are:

• surgery (taking out the cancer in an operation)
• chemotherapy (using drugs to kill cancer cells)
• radiation therapy (using high-dose x-rays to kill cancer cells)

Treatment and prognosis depends on the stage of the disease, where it is found, and the patient's age and general health.

Previously Untreated (Stages 1-4)

• If the cancer can be removed, treatment will probably be surgery followed by chemotherapy. Radiation therapy may also be given if there is cancer left following the surgery. If the cancer cannot be removed with surgery or if it is near an important organ (e.g., the eye or bladder), treatment will probably be chemotherapy plus radiation therapy, followed, if needed, by surgery.
  
  
• Clinical trials are testing new chemotherapy drugs, new ways of giving radiation therapy, and autologous bone marrow transplantation following high-dose chemotherapy

Recurrent Rhabdomyosarcoma

• Treatment depends on how much of the cancer can be removed by surgery, where the cancer came back, and the treatment given in the past. Chemotherapy may be given.
  
  
• Clinical trials are testing new chemotherapy agents and autologous bone marrow transplantation.

Clinical Trials available at Johns Hopkins

• Phase IIIdy of Dactinomycin and Vincristine With or Without Cyclophosphamide and/or Radiotherapy in Patients With Newly Diagnosed Low Risk Rhabdomyosarcoma of Embryonal or Botyroid Subtype. (IRS-D9602)
  
  
• Phase III Randomized Study of Vincristine, Dactinomycin, and Cyclophosphamide (VAC) Versus VAC Alternationg With Vincristine, Topotecan, and Cyclophosphamide in Patients With Intermediate Risk, Previously Untreated Rhabdmyosarcoma.(IRS-D9803)
  
  
• Phase II Upfront Window Study of Irinotecan Followed by Multimodal, Multiagent Therapy in Selected Children and Adolescents With Newly Diagnosed Stage IV Clinical Group IV Rhabdomyosarcoma. (IRS-D9802)
  
  
• Phase II Study of Irinotecan in Children With Refractory CNS or Solid Tumors.(P9761)

 

Other Childhood Soft Tissue Sarcomas
(Desmoid tumor, Fibrosarcoma, Mailgnant fibrous histiocytoma, Liposarcoma, Leiomyosarcoma, Angiosarcoma, Hemangiopericytoma, Synovial sarcoma, Malignant Schwannoma, Malignant Mesenchymoma, Epitheloid sarcoma, Clear cell sarcoma)

Soft tissue sarcomas are diseases in which cancer cells begin growing in soft tissue of the body. The soft tissues include muscles, tendons (bands of fiber that connect muscles to bones), fibrous (connective) tissues, fat, blood vessels, nerves, and synovial tissues (tissues around joints). Soft tissues connect, support, and surround other body parts and organs. Soft tissue sarcomas may develop in any part of the body, but in young patients they are most commonly found in the trunk, arms, and legs.

Risk Factors
The known risk factors for these types of Soft Tissue Sarcomas are:

• Congenital anomalies
• Genetic disorders such as Li-Fraumeni syndrome and neurofibromatosis.

Symptoms
The first symptom may be a solid mass or lump. If the mass interferes with a function of the body, it may cause other symptoms.

Diagnosis
If your child has symptoms, the doctor may do x-rays and blood tests. If an abnormal mass is found, a biopsy may be done. The surgeon will remove a piece of tissue from the affected area. This tissue will be examined under a microscope to see if cancer cells are present.

Prevention
There is currently no way to prevent Soft Tissue Sarcomas.

Staging
There are several staging systems for childhood soft tissue sarcoma, but no single staging system applies to all types of cancer. The treatment options are based on whether the cancer has spread or the amount of tumor left after surgery. The 3 general stages of soft tissue sarcoma are nonmetastatic, metastatic, and recurrent.

• Nonmetastatic childhood soft tissue sarcoma:
  The cancer is found only in the area where it started and has not spread to other parts of the body.
  
  
• Metastatic childhood soft tissue sarcoma:
  The cancer has spread from where it started to other parts of the body.
  
  
• Recurrent soft tissue sarcoma:
  The cancer has come back (recurred) after it has been treated. It may come back in the area where it started or in another part of the body.

Treatment
The treatments that are used to treat Soft Tissue Sarcomas are:

• surgery (taking out the cancer in an operation)
• chemotherapy (using drugs to kill cancer cells)
• radiation therapy (using high-dose x-rays to kill cancer cells)

Treatment depends on the type of soft tissue sarcoma.

Nonmetastatic Childhood Soft Tissue Sarcoma

For fibrosarcoma, hemangiopericytoma, desmoid tumor and angiomatoid malignant fibrous histiocytoma treatment may be one of the following:

• Surgery to remove all of the cancer. Sometimes a second operation must be done to be sure that all the tumor has been removed, or if the tumor comes back following treatment.
  
  
• Surgery to remove the cancer followed by radiation therapy (if a second surgery is not possible).
  
  
• Chemotherapy to reduce the tumor size, followed by surgery.

For desmoid tumor, treatment may be one of the following:

• Surgery to remove all of the tumor.
  
  
• Chemotherapy is currently recommended if complete removal of the tumor is not possible.
  
  
• Radiation may be recommended in some cases.
  
  
• Patients may undergo close monitoring when no other treatment alternatives are available and the tumor does not place any vital organs in danger (if complete removal of the tumor is not possible or if the tumor comes back following treatment).

For fibrosarcoma, hemangiopericytoma, malignant peripheral nerve sheath tumor, liposarcoma, synovial sarcoma, malignant fibrous histiocytoma, leiomyosarcoma, or epitheloid sarcoma treatment may be one of the following:

• Surgery to remove all of the cancer. Sometimes a second operation must be done to be sure that all the tumor has been removed.
  
  
• Surgery and radiation therapy (if the tumor cannot be completely removed by surgery).

For alveolar soft part sarcoma, treatment may be one of the following:

• Surgery to remove all of the cancer.
  
  
• Surgery followed by radiation therapy (if the cancer is not completely removed during surgery).

   

Metastatic Childhood Soft Tissue Sarcoma

Treatment for metastatic soft tissue sarcoma may include combined chemotherapy, radiation therapy, and surgery to remove the cancer that has spread to the lungs.

Clinical trials are evaluating the effectiveness of combination chemotherapy and colony-stimulating factors in patients who have not undergone surgery or who have metastatic soft tissue sarcoma.

Recurrent Childhood Soft Tissue Sarcoma

Treatment for recurrent childhood soft tissue sarcoma depends on the treatment given before, the part of the body where the cancer has come back and the general health of the patient. Treatment may be one of the following:

• Surgery to remove the cancer.
  
  
• Surgery followed by radiation therapy (if the patient has not yet received radiation therapy).
  
  
• Amputation of sarcomas of the arm or leg in patients who have previously received radiation therapy.

Clinical trials are evaluating chemotherapy.

 

Click here for more information on The Sarcoma Center at Johns Hopkins