Leiomyosarcoma | Diagnosis & Treatment

How is leiomyosarcoma diagnosed?

The first step in treating your child is forming an accurate and complete diagnosis. To diagnose your child's leiomyosarcoma, your specialist uses a combination of medical history, physical examination and laboratory tests.

Diagnostic procedures for leiomyosarcoma determine the exact type of tumor your child has and whether the tumor has spread. These may include a:

  • physical exam, including neurologic function tests including: reflexes, muscle strength, eye and mouth movement, coordination and alertness
  • x-rays
  • magnetic resonance imaging (MRI)
  • computerized tomography scan (also called a CT or CAT scan)
  • biopsy or tissue sample from the tumor to provide definitive information about the type of tumor
  • bone scan to detect bone diseases and tumors as well as to determine the cause of bone pain or inflammation
  • complete blood count (CBC), which measures size, number and maturity of different blood cells in a specific volume of blood
  • blood tests including blood chemistries
  • CT-guided core needle biopsy

What are the treatment options for leiomyosarcomas?

Traditional treatments for leiomyosarcoma

Surgery to remove the entire tumor, along with surrounding healthy muscle and other tissue is usually the best option in attacking leiomyosarcoma. Radiation and chemotherapy may also be used to treat some forms of leiomyosarcoma. Surgery is preferable since radiation and chemotherapy are not as effective against certain types of leiomyosarcoma, such as the type that arises from the gastrointestinal tract.

Treatment may include some combination of the following:

Surgery

Depending on the size and location of the tumor, your child may either need:

  • limb-salvage surgery, which helps preserve the limb by removing the tumor and wide margins of healthy tissue surrounding the tumor
  • amputation may be necessary if the tumor cannot be removed (for example, if it involves the nerves and blood vessels)

Surgical removal is usually the first and most important treatment option. If the tumors are, completely removed, it won't re-appear in another part of the body.

Radiation therapy

Our doctors use precisely targeted and dosed radiation to kill cancer cells left behind after your child's surgery. Radiation is necessary if surgery does not completely remove the tumor and some of the tumor is left behind.

Chemotherapy

Chemotherapy is a drug that interferes with the cancer cell's ability to grow or reproduce. Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors. Often, a combination of chemotherapy drugs is used. Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat. The main purpose of chemotherapy is to stop the cancer from metasizing, and moving to other parts of the body.

It can be used pre-operatively (neo-adjuvant) or before surgery, to clear tumors in “hard to reach” areas where there are vital organs, and make surgical removal easier. Post-operative (adjuvant), or after surgery, can be helpful for leiomyosarcoma in extremities such as fingers.

While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. In other words, the chemotherapy will attack normal healthy cells as well as the cancer cells since the chemotherapy drugs can't tell the difference. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.

What is the long-term outlook of leiomyosarcomas?

Leiomyosarcoma is extremely rare, and there are no official numbers on how well children manage the disease in the long term. However, doctors have studied soft tissue sarcomas (which is the category of cancer that and find that leiomyosarcoma falls under) and find that if a soft tissue sarcoma is completely resected, or removed, the survival rate is 80 percent or greater.