Research shows that leiomyosarcoma is the result of cell deoxyribonucleic acid (DNA) mutations. However, what causes that mutation is not entirely known. They suspect this gene mutation could be inherited from a parent/family. Some types of rare genetic conditions can increase the risk of getting leiomyosarcoma (LMS). A genetic condition can happen in a family that has not had it before (mutation), but this is rare.
- This is a genetic disease in which noncancerous (benign) tumors form in the nerves under the skin and in other parts of the body.
- It increases the risk of getting a rare type of sarcoma called a malignant peripheral nerve sheath tumor (MPNST).
Li-Fraumeni syndrome
- This is a genetic syndrome that causes several different types of cancer to occur in affected families. It’s sometimes called family cancer syndrome.
- Families with Li-Fraumeni syndrome have a higher risk of developing soft tissue sarcoma and other cancers.
- This is a type of inherited eye cancer. It is nearly always diagnosed in childhood.
- Children who have had retinoblastoma have an increased risk of developing a soft tissue sarcoma in the future.
- The most common types are leiomyosarcoma, fibrosarcoma and rhabdomyosarcoma. The radiotherapy given as treatment for retinoblastoma may partly account for the increased risk.
- These children also have an increased risk of getting a type of bone cancer called osteosarcoma.
However, genetic changes could spontaneously develop, be environmentally induced by radiation treatments at a younger age or be the result of exposures to carcinogenic agents, such as pesticides and weed killers. A breast cancer drug called Nolvadex (tamoxifen) has been linked to uterine leiomyosarcoma.
What is leiomyosarcoma (LMS)?
Leiomyosarcoma or LMS is a type of rare cancer that grows in the smooth muscles. These smooth muscles line the hollow organs of the body, such as the intestines, stomach, bladder and blood vessels. In females, there is also smooth muscle in the uterus. LMS is an aggressive cancer, which means it can grow quickly. It is found most often in the abdomen or the uterus. Leiomyosarcoma commonly spreads through the bloodstream to the lungs and liver, but at later stages, it can appear anywhere in the body. Both children and adults can develop leiomyosarcoma, although it predominantly occurs in adults aged between the ages of 40 to 60 years old. The signs and symptoms of LMS depend on the size and its location
- Bloating in the abdomen
- Tiredness
- Fever
- Lump or swelling under the skin
- Nausea and vomiting
- Pain
- Weight loss
- Black-colored stools or blood vomiting
- Bleeding from the vagina, which is not from a menstrual period
- Discharge from the vagina
- Need to urinate more often than usual
Diagnosis of leiomyosarcoma (LMS)
- Depending on the symptoms, the doctor may recommend imaging scans, such as magnetic resonance imaging (MRI), computed tomography (CT), angiography and positron emission tomography (PET), to look at the location and size of the tumor. They will also check for signs to identify if the tumor has spread to other parts of the body.
- To check if the tumor is LMS, the doctor will do a biopsy. They take a small sample from the tumor with a needle. Then the pathologist will study cells from the sample under the microscope to see what kind of tumor it is.
- Sometimes, LMS may be mistaken for a different type of tumor or condition using imaging scans. A biopsy will confirm if the tumor is LMS.
- Getting the correct diagnosis is particularly important to get the right treatment.
Treatment options for leiomyosarcoma (LMS)
- Surgery is the most common treatment for LMS. The surgeon will take out the tumor and some of the tissue around it.
- Women who have cancer in their uterus will need surgery to have the organ taken out. They may also have their fallopian tubes and ovaries removed if cancer has spread there.
- Radiation therapy: High-energy X-rays kill cancer cells or stop their growth. This is used to shrink the tumor before or after surgery.
- Chemotherapy: Medicine is used to kill cancer cells. The doctor might give chemo if the cancer has spread or comes back after treatment. The doctor might use a combination of two or more chemotherapy drugs.
- After treatment, regular checkups with the doctor are important. If cancer comes back, it will be treated again with surgery, radiation or chemotherapy.
The prognosis for leiomyosarcoma depends on the size and location of the tumor. Superficial tumors discovered early in their course are associated with a good prognosis, and there is a 5-year survival rate of approximately 90 percent. Larger tumors diagnosed later in their development can cause death by local extension and metastatic deposits. For example, the 5-year survival rate of uterine leiomyosarcoma is only 50 percent for stage I disease. The rate falls to less than 20 percent when there are metastases present outside the location of the tumor.
Liddy Shriver Sarcoma Initiative
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