• Leiomyosarcoma Overview

    Leiomyosarcoma is a very rare, but treatable form of cancer. This soft-tissue sarcoma grows in smooth muscle tissues in the skin and organs. In children, it usually grows near the gastrointestinal tract, affecting the stomach, small intestine, colon, appendix rectum or anus. These tumors are not highly aggressive and, if treated early, rarely spread or are life-threatening.

    There are several different types of leiomyosarcomas:

    • Soft-tissue leiomyosarcoma, which is usually found in the gastrointestinal tract
    • Cutaneous (skin) leiomyosarcoma, which, at the surface level, is benign and does not spread
    • Vascular leiomyosarcoma, which occurs in large veins and the vena cava (the veins the return deoxygenated blood from the body to the heart)
    • Immunocompromised host leiomyosarcoma, which occurs in children with HIV, AIDS or Epstein-Barr virus
    • Bone leiomyosarcoma, which occurs in long bones near the growth plates

    Leiomyosarcoma Treatment at Dana-Farber/Boston Children's

    Children leiomyosarcoma are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through our Bone and Soft Tissue Program. Continue reading to learn more about leiomyosarcoma or visit the Bone and Soft Tissue Program homepage to learn about our expertise and treatment options for this condition.

    Symptoms & Diagnosis

    Leiomyosarcomas may exist for a long time before they are discovered. The most common symptoms  include:

    • Painless swelling or mass anywhere in the body
    • Pain or soreness caused by compressed nerves or muscles
    • Limping or other difficulty using the legs, feet, arms or hands

    Different forms of the disease have specific symptoms, including:

    • Abdominal discomfort and weight loss (soft-tissue)
    • Purple or red spots deep in the skin (cutaneous)
    • Vein blockages, accumulated abdominal fluid, enlarged liver (vascular)

    In addition to physical and neurological examinations, leiomyosarcoma is diagnosed with:

    • X-ray
    • Magnetic resonance imaging (MRI)
    • Computed tomography (CT or CAT) scan
    • Biopsy
    • Bone scan – to determine the cause of pain and inflammation
    • Complete blood count and blood chemistry tests
    • Biopsy

    After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    Doctors treat leiomyosarcoma with:

    • Surgery – to remove the entire tumor and surrounding healthy muscle and other tissue; if the tumor is completely removed, it is unlikely to reappear elsewhere
    • Radiation therapy – if surgery does not completely remove the tumor 
    • Chemotherapy – before surgery to clear the tumor in hard-to-reach areas to make surgery easier; after surgery, it is helpful in disease affecting the extremities such as the fingers

    Progressive or Recurrent Disease

    Leiomyosarcoma can remain dormant and recur years after initial treatment. Specific guidelines for treating recurrent disease are lacking, however, because of the rare nature of the disease.

    Long-term Outlook

    If the tumor is removed early enough before it grows too large, it can be removed before it spreads elsewhere in the body. In this case, survival is 80 percent or better.
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