KidsMD Health Topics


  • If your child has been diagnosed with leiomyosarcoma, you’ll have concerns and questions about her health, treatment, recovery and other issues. It may comfort you to know that leiomyosarcoma is a very rare, but very treatable cancer, and that Dana-Farber/Boston Children’s Cancer and Blood Disorders Center is a world leader in treating children with cancer. We specialize in innovative, family-centered care that supports your child and family every step of the way.

    About leiomyosarcoma

    Leiomyosarcomais a very rare, but very treatable cancer. It’s a type of soft tissue sarcoma (cancer) that grows in muscle tissue in muscles, including muscles in the skin and organs. In children, it usually grows in the gastrointestinal tract. Since it is in the soft tissue areas, it can be difficult to detect because they are covered by other structures such as skeletal muscle.

    • Leiomyosarcoma can spread (metastasize) to other areas of the body, but usually does not if it is removed early.
    • Its cause is unknown but has been linked to genetics and inherited diseases.
    • Children with AIDS are at risk for developing this kind of cancer.
    • A tumor may exist for a long time before being discovered.
    • Treatment usually involves surgery and potentially radiation and/or chemotherapy.
    • Leiomyosarcoma is very treatable, with 80% success rate if the tumor is completely removed.
    • There are no gender related risks among children. Girls and boys have equal risks of having leiomyosarcoma.
    • It is extremely rare. There are only 20 to 30 cases of child leiomyosarcoma cases per year in the United States.
    • The outlook is very good if the tumor is completely removed.

    How Dana-Farber/Boston Children’s Cancer and Blood Disorders Center approaches leiomyosarcoma

    Dana-Farber/Boston Children’s Cancer and Blood Disorders Center provides compassionate, comprehensive medical and surgical care for children and adolescents with benign or malignant soft tissue tumors.

    Our multidisciplinary approach to care ensures in-depth discussion of each case and personalized treatment plans for every patient. We integrate expertise from the following specialists:

    In addition to a multidisciplinary approach, we are starting a personalized medicine approach to make sure we treat each leiomyosarcoma case separately, as each person is unique, down to the molecular level. We develop a treatment plan specifically designed for your child.

    Leiomyosarcoma: Reviewed by Carlos Rodriguez-Galindo, MD © Boston Children’s Hospital; posted in 2012

    Boston Children's Hospital
    300 Longwood Ave
    Fegan 2
    Boston MA 02115 

    fax: 617-730-0456 

  • In-Depth

    Here at Children’s Hospital Boston, we specialize in innovative, family-centered care. From your first visit, you’ll work with a team of professionals who are committed to supporting all of your family’s physical and psychosocial needs. We understand that you probably want to learn all you can about your child’s leiomyosarcoma to determine your next steps.  

    What is leiomyosarcoma?

    Leiomyosarcoma is a cancer of the muscle, particularly of soft tissue. It is a type of soft tissue sarcoma, and can appear in many places in the body, but in children it usually occurs in the gastrointestinal tract, which includes the stomach, small intestines, colon, appendix, rectum and anus. It can spread (metasize) to other areas of the body if it’s not removed, but if it is completely removed, it almost never reappear in other parts of the body. Overall, it is not a highly aggressive form of cancer and usually is not considered life threatening if it is treated early.

    What are the different kinds of leiomyosarcoma?

    • soft tissue leiomyosarcoma
      • In children, soft tissue leiomyosarcoma is usually found in the gastrointestinal tract, which includes the stomach, small intestines, colon, appendix, rectum and anus.
      • Symptoms include abdominal discomfort and weight loss.
    • cutaneous (skin) leiomyosarcoma
      • Men are twice as likely to develop cutaneous leiomyosarcoma. In children, the risk is equal among girls and boys.
      • Surface level cutaneous leiomyosarcoma is usually benign, and usually does not move to other parts of the body (metastasize).
      • Small purple or red spots (lesions) deeper in the skin
    • vascular leiomyosarcoma
      • originating from major blood vessels
      • very rare condition
      • only a few hundred cases reported, ever
      • occurs in:
        • larger veins
        • vena cava
          • Veins that carry “old” blood that lacks oxygen (deoxygenated blood) from the body to the heart to be pumped through and regain oxygen.
          • Tumors in the vena cava cause Budd-Chiari syndrome, which leads to:
            • blockage of the vein (occlusion)
            • abdominal pain
            • accumulated abdominal fluid (acites)
            • enlarged liver (hepatomegaly)
            • yellow discoloring of the skin (jaundice)
        • pulmonary artery
          • shortness of breath, chest discomfort
    • immunocompromised host leiomyosarcoma
      • HIV, AIDS and the Eptstein-Barr virus (“mono”) compromises the immune system and can make your child more vulnerable to leiomyosarcoma.
    • bone leiomyosarcoma
      • extremely rare
        • only 90 cases reported since 1965
      • occurs in the metaphysis of long bones, where epiphyseal plates (“growth plates”) are
        • “Growth” plates start as cartilage and become bone (ossifies) when the child has stopped growing.
      • tumors in the long bones hard to find, as they appear translucent in radiographic testing (x-rays)

    Is leiomyosarcoma common?

    No, they are rare. As a group, soft tissue sarcomas (other than rhabdomysarcoma, which is slightly more common) account for less than 1 percent of all new cancer cases each year and 3 percent of all childhood tumors. More specifically:

    • Leiomyosarcoma accounts for 7 to 11 percent of all soft tissue sarcomas among adults and children.
    • In children, leiomyosarcoma is the most commonly found in the gastrointestinal tract.

    Will my child be OK?

    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.  

    Is it curable?

    Yes. If the tumor is recognized and removed early enough before it grows too large, it can be resected and removed before it metastasizes, or moves to other regions of the body.

    Symptoms and Causes

    What are the symptoms of leiomyosarcoma?

    Because soft tissue sarcomas affect tissue that is elastic and easily moved, a tumor may exist for a long time before being discovered, growing large and pushing aside surrounding tissue. Symptoms vary greatly with the size, location and spread of the tumor, but may include:

    • painless swelling or mass anywhere on the body
    • pain or soreness caused by compressed nerves or muscles
    • limping or other difficulty using the legs, feet, arms or hands

    When are symptoms noticed?

    If a child has leiomyosarcoma, usually the symptoms do not appear until adolescence, since it usually occurs in adults.

    What causes leiomyosarcoma?

    The exact cause of leiomyosarcoma is not entirely understood. However, studies have indicated that genetics may play a role in the formation of all soft tissue sarcomas. In addition:

    • Researchers have studied a small number of families that contain several members of one generation who have developed soft tissue sarcomas.
    • Limited studies have shown a possible link between soft tissue sarcomas and the development of other types of cancer.
    • Some inherited diseases have also been linked with an increased risk of developing soft tissue sarcomas.
    • There seems to be a link between leiomyosarcoma and Epstein-Barr virus infection.
    • Children with AIDS and those who are undergoing immunosupression therapy for solid organ transplantation are also at increased risk for developing leiomyosarcoma.
    • Children with other forms of cancer, such as retinoblastoma, may be at higher risk for developing leiomyosarcoma as adults.

    Questions to ask your doctor

    After your child is diagnosed with leiomyosarcoma, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you.

    Lots of parents find it helpful to jot down questions as they arise- that way, when you talk to your child’s doctors you can be sure that all of your questions are concerned. If your child is old enough, you may want to suggest that he writes down what he wants to ask her health care provider too.

    Here are some questions to get you started:

    • What can I do to help my child get ready for surgery?
    • Are there any particular foods or drink my child should avoid?
    • Could any of the current medications my child is taking affect her condition?
    • What are some ways I can address my child’s fear of surgery, chemotherapy or radiation?
    • How will I know if the tumor is completely removed?
    • How can I help my child recover after surgery?
    • When will my child be able to resume normal physical activity?
    • How could treatment affect my child’s academic and social life?
      Glossary of useful terms

      Have questions about some of the terms mentioned on the page? Visit our Cancer Care Glossary for more information.

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

    How does a doctor know that it’s leiomyosarcoma?

    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, which produce images of internal tissues, bones and organs onto film
    • magnetic resonance imaging (MRI), which produces detailed images of organs and structures within the body and/or spine
    • computerized tomography scan (also called a CT or CAT scan) to capture a detailed view of the body, in some cases
    • biopsy or tissue sample from the tumor to provide definitive information about the type of tumor; other is collected during surgery
    • 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
      • less invasive than an incisional biopsy

    What is differentiation (hertological grading)?

    Differentiation is how much a cell stands out from surrounding cells.

    For example, a fat cell looks completely different than a cartilage cell.

    In terms of leiomyosarcoma, differentiation is about how different the tumor cells appear from the normal cells it originated from.

    For example, if it is a leiomyosarcoma of soft tissue, it’s a matter of how different the tumor may look from the soft tissue.

    Highly differentiated tumors look like the cells they originated from.

    Low differentiated tumors look very different from the cells they originated from.

    The less differentiated the tumor is, the more mitosis is going on, meaning the tumor cells are dividing and growing.

    What is grading of tumors?

    • Grading is a way to identify the severity of the tumor, and helps to identify the appropriate treatment.
    • There are three grades: low, intermediate and high.
      • Low grade means it is more differentiated, local and usually harmless (benign).
      • High grade means it is undifferentiated, growing and usually cancerous.
    • Ideally, you want a low grade tumor which is highly differentiated, with little or no growth and is benign.

    After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child’s condition. Then we meet with you and your family to discuss the results and outline the best treatment options.

  • It's entirely natural that you are concerned right now about your child's health; a diagnosis of leiomyosarcoma can be frightening. But you can be rest assured that, at Children's, your child is in good hands. Our physicians are bright, compassionate and committed to focusing on the whole child, not just her condition – that's one reason we're frequently ranked as a top pediatric hospital in the United States.

    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:


    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 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.
    • 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.

    Chemotherapy is a systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:

    • as a pill to swallow
    • as an injection into the muscle or fat tissue
    • intravenously (IV), directly to the bloodstream

    What is the recommended long-term care for children treated for leiomyosarcoma?

    Children treated for leiomyosarcoma should visit a survivorship clinic every year to:

    • manage disease complications
    • screen for early recurrence of cancer
    • manage late effects of treatment

    A typical follow-up visit may include some or all of the following:

    • a physical exam
    • laboratory testing
    • imaging scans

    Through the David B. Perini, Jr. Quality of Life Clinic at Dana-Farber Cancer Institute, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team.

    • Our childhood cancer survivorship clinic is held weekly.
    • In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists or alternative/complementary therapy specialists.
    • We also offer the following services:
      • patient and family education
      • psychosocial assessment
      • genetic counseling
      • reproductive and fertility evaluation and counseling
      • opportunities to speak with other childhood cancer survivors

  • Dana-Farber/Boston Children’s Cancer and Blood Disorders Center is the world’s largest research program at a pediatric institution, and we’re known for pioneering new treatments. A large part of our success comes from our commitment to research—and to advancing the frontiers of what’s possible through our innovative approach.


    Boston Children's Hospital and Dana-Farber Cancer Institute are also conducting numerous research studies to help better understand and treat soft tissue sarcomas.

    Research underway

    Types of treatment currently being studied include:

    • angiogenesis inhibitors, substances that may be able to prevent the growth of tumors
    • biological therapies, a wide range of substances that may be able to involve the body's own immune system to fight cancer or lessen harmful side effects of some treatments

    OncoMap: A personalized approach to treatment

    OncoMap is a new approach of mass spectrometry (a method of looking at genes at a molecular level) used at the Dana-Farber/Boston Children’s Cancer and Blood Disorders Center. Mass spectrometry is a screening technology typically used for detecting changes in genetic material, DNA. OncoMap is using that same technology to look at the genetic material of tumors, essentially looking at the genetic code of the tumor and revealing information about the tumor that could help create treatment plans that can be personalized to the genetic characteristics of the patient’s tumor. To use a sports analogy, it would be like seeing the playbook of your opponent which would reveal vulnerable places to attack your opponent. Similarly, OncoMap can provide genetic information about the tumor and could help create a unique treatment plan to attack the cancer in the most effective way.

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