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Bone and Soft Tissue Tumors (Sarcomas)

Bone and soft tissue tumors can form from parts of the body’s connective tissue, such as bone, cartilage and fat.  These tumors are often benign, meaning they are not cancer and do not have the ability to spread to other parts of the body.  Malignant, or cancerous, bone and soft tissue tumors are called sarcomas.  Several of the more common sarcomas in children include osteosarcoma, Ewing’s sarcoma, rhabdomyosarcoma and chondrosarcoma.  At Ochsner Hospital for Children, our Pediatric Oncologists work with a multidisciplinary team to provide state-of-the-art care to children and young adults diagnosed with bone and soft tissue tumors.  This includes pediatric oncologists, oncologic orthopedic surgeons, radiation oncologists, surgeons, and physical therapists.  We are very active in research and clinical trials designed to increase cure rates, decrease treatment-related side effects and improve care for long-term survivors.

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What are bone and soft tissue tumors?

Bone and soft tissue tumors arise from parts of the body’s connective tissue, such as bone, cartilage and fat.  When these tumors are malignant (cancerous), they are known at sarcomas.  There are many different subtypes of bone and soft tissue tumors, depending on which type of tissue the tumor comes from.  The most common malignant subtypes include osteosarcoma, Ewing’s sarcoma, rhabdomyosarcoma and chondrosarcoma.  The different tumor types may require substantially different treatment.   

What are the symptoms of bone and soft tissue tumors?

  • Symptoms of bone and soft tissue tumors include:
  • Painless swelling in any area of the body
  • Bone pain
  • Joint pain
  • Limp or decreased use of an arm or leg
  • Fracture in a bone from minor trauma

How common are bone and soft tissue sarcomas?

Overall, these tumors are rare in children, accounting for less than 10% of all pediatric cancers.  The most common type, osteosarcoma, affects around 500 children in the US per year, while Ewing’s sarcoma affects around 200 patients per year.  The other types of these tumors are even more rare.  Osteosarcomas and Ewing’s sarcomas tend to be most common in teenagers, while other type of these tumors can occur at any age.

What are the survival rates for bone and soft tissue sarcomas?

  • Survival rates for these types of tumors depend on the subtype and the extent of spread of the disease
  • For osteosarcoma, localized (non-metastatic) disease has a long term survival of ~75%, while metastatic disease has a survival of ~30%
  • For Ewing’s sarcoma, ~70% of children can be cured, however children with metastatic disease at diagnosis have a lower survival rate of ~30%

How are bone and soft tissue sarcomas diagnosed?

Depending on the type and site of the tumor, a variety of techniques may be used to diagnose and stage bone and soft tissue sarcomas.  These include:

  • Biopsy, which is a surgical procedure to take a sample of the tumor in order to determine what type of tissue it is and to run special molecular tests.
  • X-rays to evaluate tumors of the bone
  • Magnetic resonance imaging (MRI), which uses strong magnets and a computer to produce detailed images of soft tissues, is useful to evaluate soft tissue sarcomas.
  • Computed tomography (CT) scan, which uses a combination of X-rays and computer to produce images of the body, is useful for evaluating both bones and soft tissues.
  • Radioisotope bone scan, which uses a special “tracer” to evaluate for abnormal areas of bone.
  • Positron emission tomography (PET) scan, which uses radioactive sugar injected into a vein to determine if the tumor has spread to other areas of the body.
  • Lumbar puncture, in which a sample of fluid is taken from the base of the spine, is used for certain subtypes of tumors that occur in the head.
  • Bone marrow biopsy, in which a small sample of bone marrow is obtained from the hip, is often performed to determine if the tumor has spread to the bone marrow.

How are bone and soft tissue sarcomas staged?

The staging of bone and soft tissue sarcomas depends upon the subtype of the tumor and can be somewhat complicated.  In general, the size, number and location of the tumors is combined with the pathology results from the tumor biopsy, bone marrow biopsies and the molecular studies and a stage and risk category is assigned.  An important distinction for all subtypes is whether the disease is “localized” (confined to the area of the body in which it started) or “metastatic” (spread to other areas of the body).

More detailed information on staging of particular subtypes of tumor can be found here:

How bone and soft tissue sarcomas treated?

Treatments for bone and soft tissue sarcomas may involve a combination of therapies including surgery, radiation and/or chemotherapy. In most cases, children receive chemotherapy before surgery (neoadjuvant), followed by surgery to remove the tumor, additional chemotherapy after surgery (adjuvant). Treatment options may vary greatly, depending on your child's situation. Our pediatric oncologists work closely with a multidisciplinary team to create a personalized treatment plan that is best for your child. 

Surgery - Depending on the size and location of the tumor and whether the tumor has spread, your child may receive one of the following surgical treatments:

  • Limb-sparing surgery involves removing the entire tumor, while still preserving as much healthy tissue surrounding the tumor as possible.  This type of surgery is done whenever possible as long as the tumor can be completely remove. The goal of limb-sparing surgery is to preserve as much use of the limb as possible and maximize long-term limb function.
  • Amputation may be necessary if the tumor cannot be completely removed (for example, if it involves the nerves and blood vessels) or if limb function cannot be preserved through limb-sparing surgery. If amputation is necessary, your child may be fitted for a prosthesis following surgery.
  • If the tumor is in areas outside of the arms and legs, a surgical resection of the tumor may be performed after completion of neoadjuvant chemotherapy
  • If the tumor has spread to other parts of the body, such as the lungs, additional surgery may be required, and our oncologic surgeons may need to remove any metastases that may have formed.

Chemotherapy - To decrease the size of the tumor, kill tumor that has spread and prevent further tumor spread, your child may receive chemotherapy. 

Radiation therapy - High-energy X-rays or other types of radiation can kill cancer cells or stop them from growing.  Radiation therapy is used in certain types of tumors to either shrink the tumor prior to surgery, or to try to prevent the tumor from coming back after surgery.

Targeted therapy - Newer medicines are now available that target and attack specific cancer cells without harming normal cells.  Many of these medicines are still considered experimental.

Rehabilitation - Rehabilitation after surgery is an important part of your child’s care. Our team of physical and occupation therapists, along with our Child Life Specialists, will work to help your child optimize their mobility after surgery and adapt to whatever types of lifestyle changes may be necessary.

What clinical trials does Ochsner Hospital for Children offer for bone and soft tissue sarcomas?

  • As part of the Children’s Oncology Group, Ochsner Hospital for Children is an active participant in children’s cancer research.  We currently have multiple clinical trials available to treat children and young adults with various types of bone and soft tissue sarcomas.