Leiomyosarcoma

What is leiomyosarcoma?

Leiomyosarcoma (LMS) is a rare, aggressive cancer that forms in smooth muscles. Smooth muscles are involuntary muscles located in various parts of your body. You have smooth muscles in your hollow organs, including your:

• Bladder
• Blood vessels
• Large intestine
• Small intestine
• Stomach
• Uterus

LMS cancer is a type of soft tissue sarcoma. It grows quickly and can double in size in as little as one month. The cancer cells travel through your bloodstream and can spread to any soft tissue in your body.

Some people don’t develop LMS symptoms until the disease reaches an advanced stage. In these cases, leiomyosarcoma is life-threatening. But when it’s detected and treated early, recovery is possible.

A cancer diagnosis can be overwhelming. Lean on your healthcare team. They can recommend resources and support groups that may help you on your journey.

Types of leiomyosarcoma

There are three subtypes of leiomyosarcoma:

• Somatic soft tissue LMS. This affects your connective tissue. It’s the most common form of LMS. Uterine leiomyosarcoma is one example of somatic soft tissue LMS.
• Cutaneous or subcutaneous LMS. This involves piloerector muscles in your skin and eyes. Your piloerector muscles give your skin goosebumps and make your pupils dilate.
• LMS of a vascular origin. This forms in a major blood vessel, like your pulmonary arteries, inferior vena cava or peripheral arteries. It’s the rarest form of LMS.

How common is leiomyosarcoma?

In the United States, about 15,000 people receive a soft tissue sarcoma diagnosis every year. Leiomyosarcoma accounts for 10% to 20% of those cases. About 1 in every 100,000 people in the U.S. develops LMS cancer.

Leiomyosarcoma can affect anyone. But it’s most common in females over age 5.

What are the symptoms of leiomyosarcoma?

Leiomyosarcoma symptoms vary depending on the size and location of the tumor. Some people don’t experience symptoms early on, but may notice certain signs as the tumor grows, like:

• A firm, painless lump
• Abdominal bloating
• Fever
• Nausea and vomiting
• Pain
• Tiredness
• Weight loss

Leiomyosarcoma in your digestive system may cause:

• Abdominal pain
• Black stools (from blood in your poop)
• Loss of appetite
• Nausea and vomiting

Uterine leiomyosarcoma can cause:

• Abnormal uterine bleeding
• Frequent urination
• Vaginal discharge

What causes leiomyosarcoma?

Experts aren’t exactly sure what causes leiomyosarcoma. It could be hereditary (meaning you inherited altered genes from your parents), or it could be because your own genes changed, causing normal cells to grow out of control and become cancer cells.

Researchers have found links between LMS and these genetic conditions:

• Gardner syndrome
• Gorlin syndrome
• Hereditary retinoblastoma
• Li-Fraumeni syndrome
• Neurofibromatosis type 1 (NF1)
• Tuberous sclerosis
• Werner syndrome

How is leiomyosarcoma diagnosed?

A healthcare provider will do a physical examination and ask you about your symptoms. They’ll also review your medical history, including any past or current health conditions.

Your provider will take imaging tests to see inside your body and determine the size and location of the tumor. These imaging tests may include:

• Angiography
• Computed tomography (CT) scans
• Magnetic resonance imaging (MRI)
• PET scan

Your provider will likely need to do a biopsy, too. When testing for LMS, providers try to take small samples from several parts of the cancer. Once they have the tissue samples, they’ll send them to a pathologist for testing.

How is leiomyosarcoma treated?

Leiomyosarcoma treatment depends on the location and size of the tumor. Options include:

• Surgery. When surgery is possible, it’s the go-to treatment option for leiomyosarcoma. The goal is to remove the entire tumor so that the cancer doesn’t come back.
• Chemotherapy. Providers recommend chemotherapy when the tumor is large, or when cancer cells have spread to other parts of your body.
• Radiation therapy. Providers might use radiation therapy before surgery (neoadjuvant therapy) to shrink the tumor, or after surgery (adjuvant therapy) to kill any remaining cancer cells.
• Targeted therapy. Your healthcare team may recommend targeted therapy as a stand-alone therapy or in combination with other treatments.

How long does it take to recover from leiomyosarcoma treatment?

Recovery times can vary drastically depending on several factors, including:

• The size and location of the cancer
• The type of treatment you receive
• Your body’s healing capacity

It could take several weeks or months to fully recover. Even after you’re feeling better, you’ll still need regular checkups to monitor your health and reduce the risk of cancer recurrence (return).

Can leiomyosarcoma be prevented?

Currently, there’s no known way to prevent leiomyosarcoma. But you can reduce your risk by avoiding risk factors whenever possible. Known leiomyosarcoma risk factors include:

• Certain viral infections, like human herpesvirus 8 (HHV8)
• Radiation exposure
• Tamoxifen (commonly used to treat breast cancer)

What’s the outlook for leiomyosarcoma?

The outlook for leiomyosarcoma varies significantly depending on the stage, size and location of the tumor. In some cases, LMS is curable, especially when detected and treated early.

Treatment is more complicated when it’s discovered in the later stages. Advanced, Stage 4 leiomyosarcoma can be managed with treatment, but not cured.

What is the survival rate of leiomyosarcoma?

Leiomyosarcoma survival rates depend on several factors like:

• Tumor size and location
• How much of the tumor your surgeon can remove
• Whether the cancer has spread — and if so, how far

The following shows five-year survival rates for three different stages of LMS (localized, regional and distant):

Survival rates are only estimates. Researchers base them on the experiences of people who had LMS in the past. Survival rates can’t tell you how long you’ll live or how well you’ll respond to treatment.

To learn more about survival rates and what they mean for you, talk to your healthcare provider.

When should I see my healthcare provider?

If you’re undergoing treatment for leiomyosarcoma, call your healthcare provider whenever you notice new or worsening symptoms. For example, if you notice any changes to your tumor — or if you develop severe pain, sudden weight changes or other symptoms — seek prompt medical care.

What questions should I ask my doctor?

If you have leiomyosarcoma, talking with your healthcare provider can inform, empower and help you take control of your health. Here are some questions you may want to ask:

• Where’s my cancer located?
• Has my cancer spread?
• How advanced is my cancer?
• What are my treatment options?
• What are the risks and side effects of treatment?
• What are the chances that my cancer will come back after treatment?
• What’s my outlook?