What Is the Survival Rate for Leiomyosarcoma?

Leiomyosarcoma is a type of soft tissue cancer that develops in the smooth muscles. Smooth muscles are involuntary muscles that line internal organs such as the gastrointestinal tract, bladder and blood vessels, and help them function. Leiomyosarcoma most often develops in the abdomen, uterus, arms or legs but can start anywhere in the body.

Leiomyosarcoma is a rare and aggressive cancer that often recurs after treatment and spreads (metastasizes) to other parts of the body. Cancers are a group of diseases in which abnormal cells grow and proliferate out of control and often form lesions or tumors.

Symptoms of leiomyosarcoma depend on the size, location and stage of the cancer. There may be no symptoms in the early stages, and initial signs may be general cancer symptoms such as:

• Fatigue
• Fever
• General malaise
• Nausea and vomiting
• Unexplained weight loss

In later stages symptoms of leiomyosarcoma can include:

• A palpable lump under the skin or unusual swelling with or without pain
• Abdominal bloating and swelling
• Trouble breathing

If the tumor is in the GI tract, symptoms include:

• Abdominal pain
• Black, tarry, foul-smelling stools due to GI bleeding
• Vomiting blood

Symptoms of leiomyosarcoma in the uterus include:

• Abnormal vaginal bleeding
• Abnormal vaginal discharge
• Change in bladder and bowel habits

Leiomyosarcoma, like other cancers, occurs due to genetic mutations in the cells. Risk factors for developing leiomyosarcoma include:

• Inherited genetic conditions with increased risk for cancers, such as:
  • Neurofibromatosis
  • Hereditary retinoblastoma
  • Li-Fraumeni syndrome
  • Gardner syndrome
  • Werner syndrome
  • Tuberous sclerosis
  • Nevoid basal cell carcinoma syndrome (Gorlin syndrome)
• Advanced age; most cases occur after the age of 50
• Radiation therapy for other cancers
• Exposure to carcinogenic chemicals such as vinyl chloride, dioxins and herbicides
• Viral infections from human herpesvirus-8 and human immunodeficiency virus (HIV)
• Immunosuppressive medications
• Hormonal disorders
• Autoimmune diseases
• Swelling (edema) in the arms after mastectomy for breast cancer
• Chronic inflammatory conditions such as ulcers
• Exposure to cyclophosphamide, a cancer medication
• Treatment with a power morcellator, a surgical device that is used to break up and remove large fibroids in the uterus, which can break up undetected uterine leiomyosarcoma and spread it

Currently, there is no cure for leiomyosarcoma. The chance for remission is best if the tumor is low grade and is diagnosed at an early stage, but leiomyosarcoma is an aggressive cancer that is often diagnosed at later stages, when it has spread to other parts of the body.

The tumor must be surgically removed with a wide margin of healthy tissue to make sure no cancer cells are left behind, but this may not always be possible, especially if the tumors are in deep tissues. Additional treatments like radiation, chemotherapy and other medical therapies can help control leiomyosarcoma.

The treatment for leiomyosarcoma is individualized based on the size, grade, stage and location of the tumor. In addition to standard treatments, it is also possible to enroll in clinical trials for new treatments. Standard treatments for leiomyosarcoma typically include a combination of the following:

Surgery

The primary treatment for leiomyosarcoma is surgical removal of the tumor wherever possible, whether it is an isolated tumor or metastatic tumors. If the tumor is in one of the limbs, it is typically removed with wide margins and a tissue graft may be used to replace the removed tissue. Nearby lymph nodes may also be removed, if the tumor has spread to them.

If significant blood vessels, nerves and muscles have to be removed because of the tumor, it can affect the limb function or cause chronic pain. In such cases limb amputation may be an option. If the tumors have spread to other organs such as lungs, they are removed to the extent possible.

Radiation and chemotherapy are often used after surgery to destroy cancer cells, particularly when it is not possible to remove the tumor with wide margins. Radiation and/or chemotherapy may sometimes be used to shrink large tumors before surgery.

Radiotherapy

Radiotherapy is the use of high-energy rays or particles directed at the tumor cells to destroy them. X-rays or proton streams are used in radiation therapy; however, proton therapy is not widely available. Radiation is the primary treatment when surgery is not possible for any reason.

Radiation may be given externally with the X-ray beams directed at the tumor, or internally (brachytherapy) for uterine leiomyosarcoma. The surgeon may also give a large dose of radiation directly at the site of the tumor after its removal, before closing the wound.

Medical therapy

Medical therapy for leiomyosarcoma is a systemic treatment useful in advanced cancers that have spread. Medical therapies include:

• Chemotherapy: Chemotherapy is the use of medications that are toxic to all cells that are in the phase of growth and division. Chemotherapy is lethal to cancer cells because they are constantly growing and dividing.
• Targeted therapy: Targeted therapy medications do not kill cancer cells, but slow down tumor growth by blocking the activity of specific proteins that help tumors grow. Certain targeted therapy methods also prevent new blood vessel formation in tumors, starving them of oxygen and nutrients.
• Immunotherapy: Immunotherapy medications enhance the patient’s own immune system’s ability to recognize and attack the cancer cells.