Leiomyosarcoma (LMS) is a rare type of cancer that develops in smooth muscle cells.
When you come to AHN for care, you will be treated by experienced, compassionate oncologists who specialize in working with sarcoma patients — specifically leiomyosarcoma. Our comprehensive treatment options include those that avoid bigger, more invasive surgeries. We also provide palliative care to effectively manage side effects throughout your care. When you’re facing a leiomyosarcoma diagnosis, AHN will provide you with the personalized treatment plan you need in a setting designed around coordinated medical care.
Leiomyosarcoma (LMS) is a rare type of cancer that affects the body’s soft tissues. "Leiomyo" refers to smooth muscle, which is found in many parts of your body, such as your:
- Uterus: The most common location for leiomyosarcoma in women.
- Abdomen: Often starting in the lining of the digestive system or the walls of blood vessels.
- Arms and legs: Can develop in the walls of blood vessels or within the muscles.
- Other areas: Less commonly, it can start in the skin, kidneys, or behind the abdomen.
It accounts for less than 1% of all soft tissue sarcomas, which themselves are relatively uncommon. The exact incidence of LMS is difficult to determine, but experts estimate that only a few thousand cases occur in the United States each year. LMS is a subtype of soft tissue sarcoma, a type of cancer that occurs in soft tissues like muscle, fat, and fibrous tissue.
AHN Sarcoma Center of Excellence
The AHN Sarcoma Center of Excellence is made up of a team of multiple health care experts walking you through every step of your cancer diagnosis and treatment.
Diagnosis and treatment will usually begin with an appointment with our orthopaedic oncology team. The team works closely with providers amongst different medical specialties including:
- Surgeons
- Medical oncology
- Radiation oncology
- Pathology
- Physical therapy
- Occupational therapy
- Plastic surgery
- Nurse Navigators
At AHN, we strive to make your treatment course as painless as possible. We will take care of appointment scheduling, imaging planning, and other aspects of care coordination for our patients so you have time to focus on healing and recovery.
Leiomyosarcoma symptoms and signs
Leiomyosarcoma is a rare type of cancer that develops in the smooth muscle tissue. Smooth muscles are found in many parts of the body, including the uterus, stomach, intestines, bladder, and blood vessels. Because of this, symptoms can vary widely depending on where the cancer develops. Many people with leiomyosarcoma might not experience any noticeable symptoms in the early stages. When symptoms do appear, they often depend on the location and size of the tumor:
- Lump or swelling: A new or growing lump that you can feel under the skin is a common sign, particularly if the tumor is located in the arms, legs, or abdomen. It might or might not be painful.
- Abdominal pain or pressure: Tumors in the abdomen can cause vague abdominal pain, a feeling of fullness, or pressure.
- Changes in bowel or bladder habits: Depending on where the tumor is located in the abdomen, you may experience constipation, diarrhea, or changes in urination.
- Uterine bleeding: In women, leiomyosarcoma in the uterus can cause abnormal vaginal bleeding or pelvic pain.
- Other general symptoms: In some cases, people may experience more general symptoms like fatigue, weight loss, or fever, but these are less specific to leiomyosarcoma and can be related to many other conditions.
Causes and risk factors
The exact causes of leiomyosarcoma aren’t fully understood, but research has identified some factors that may increase the risk of developing this cancer:
- Genetics: Some genetic syndromes, such as retinoblastoma, tuberous sclerosis, and Li-Fraumeni syndrome, can increase the risk of developing various cancers, including leiomyosarcoma. These syndromes are usually caused by inherited gene mutations.
- Radiation exposure: Having radiation therapy for a previous cancer may increase the risk of developing leiomyosarcoma in the treated area years later.
- Lymphedema: Chronic lymphedema (swelling caused by a buildup of lymph fluid) may be associated with an increased risk of developing a type of leiomyosarcoma.
- Age: Leiomyosarcoma is more common in adults, particularly older adults.
- Previous cancer treatment: As mentioned above, prior radiation therapy can increase the risk.
- Viruses: Some research suggests that infection with certain viruses, such as Epstein-Barr virus or HIV, may increase the risk.
It’s important to remember that these symptoms, signs, and risk factors don’t necessarily mean you have leiomyosarcoma. Many other conditions can cause similar symptoms, and risk factors don’t mean a diagnosis is certain. However, it’s always a good idea to get any new or persistent symptoms checked out by a doctor, and if you are more at risk, regular discussion with your doctor can help. Early detection can improve treatment outcomes.
Leiomyosarcoma screening and diagnosis
Leiomyosarcoma (LMS) is a type of cancer that arises from smooth muscle cells in various organs throughout the body. Unfortunately, there are currently no established screening tests for LMS. There are, however, different exams and tests that can help your AHN Care Team determine if there is a presence of LMS. There are a number of imaging tests your AHN doctor may order. These could include:
- X-rays: These can help identify tumors in bones or other areas, but they aren’t always effective for soft tissue tumors.
- Computed Tomography (CT) Scan: CT scans use X-rays to create detailed cross-sectional images of the body. They can help locate tumors, determine their size and shape, and see if they have spread to other areas.
- Magnetic Resonance Imaging (MRI): MRI uses radio waves and a strong magnetic field to create detailed images of the body’s soft tissues. MRI is often better than CT scans for visualizing soft tissue tumors like LMS.
- Ultrasound: Ultrasound uses sound waves to create images of the body’s internal structures. It can be useful for evaluating tumors close to the surface of the body.
- Positron Emission Tomography (PET) Scan: A PET scan uses a radioactive tracer to detect areas of increased metabolic activity, which can indicate the presence of cancer. It’s often combined with a CT scan (PET/CT) to provide both anatomical and functional information.
- Angiogram: To examine blood vessels.
Types and stages of leiomyosarcoma
LMS is classified based on its location, microscopic appearance, and sometimes its underlying genetic characteristics. While these different categories may seem confusing, this is to give you background information that can help aid in your conversation with your AHN oncologist. Your oncologist and AHN Care Team are here to clarify anything and guide you through your staging.
Where LMS starts (classification by location)
Knowing where the cancer started is the most important thing. Here are the main places LMS can develop:
- Uterine LMS (in the uterus/womb): This is the most common type. It might cause unusual vaginal bleeding, pelvic pain, or enlargement of the uterus.
- Soft Tissue LMS: This develops in the smooth muscles in your arms, legs, abdomen, or pelvis. It often feels like a lump or swelling that’s getting bigger. Sometimes, if it’s deep inside, it can grow quite large before you notice any symptoms.
- Vascular LMS (in blood vessel walls): This is rare and starts in the walls of your arteries or veins. Symptoms depend on which blood vessel is affected, and could include pain, swelling in your limbs, or even stroke-like symptoms.
- Gastrointestinal LMS (in the digestive tract): This starts in the smooth muscles of your esophagus, stomach, intestines, colon, or rectum. Symptoms will depend on the location and might include stomach pain, bleeding, or blockages.
- Cutaneous LMS (in the skin): This is very rare and grows in the smooth muscles attached to hair follicles or in blood vessels in the skin. It appears as a slow-growing bump.
- Rare locations: Very rarely, LMS can start in other places like the bladder, prostate, or adrenal gland.
What LMS looks like (classification by microscopic appearance — histology)
When doctors look at the cancer cells under a microscope (this is in your pathology report), they might describe the LMS as:
- Epithelioid leiomyosarcoma: The cells look a bit like other types of cells, which can make it harder to diagnose.
- Myxoid leiomyosarcoma: These cells contain areas of myxoid (gelatinous) deterioration.
- Pleomorphic leiomyosarcoma: This means that the cells are extremely varied in appearance.
Classification by underlying genetic characteristics
Doctors may do some genetic testing to find mutations and alterations in the cancer cells. For example, some LMS tumors may have mutations in genes like TP53, RB1, or ATRX. The presence of an NTRK gene fusion, while rare, is important to identify because it can make the tumor susceptible to targeted therapy with NTRK inhibitors. This can sometimes help with diagnosis, figuring out how the cancer might behave, and deciding on the best treatment.
Leiomyosarcoma staging
After a diagnosis, your AHN oncologist will stage the cancer. Staging takes into account how far the cancer has spread, the size of the tumor, and if it is found in the lymph nodes. In the early stages, classified as stage I or II, the tumor is generally smaller and hasn’t spread to lymph nodes or other parts of the body. Treatment often involves surgery. At later stages, III and IV, the tumor might be larger, or it may have spread to lymph nodes or other parts of the body. Treatment may involve surgery, radiation, chemotherapy, or a combination of treatments. Doctors also look at the ‘grade’ of the cancer cells — low or high — which tells how quickly they might grow and spread. The stage and grade give your doctors a roadmap to decide the best treatment options for your specific situation.
Leiomyosarcoma treatment
Treatment for leiomyosarcoma typically involves a combination of approaches, which your doctor will tailor to your specific situation. AHN’s skilled oncologists and cancer specialists will work with you to determine the right treatment course that will meet your specific health needs.
Surgery
Surgery is often the primary treatment for leiomyosarcoma, and its goal is to remove the tumor and any affected tissue while sparing as much healthy tissue as possible.
The specific type of surgery will depend on the size, location, and stage of the tumor, as well as your overall health. Common types include:
- Wide local excision: This involves removing the tumor along with a margin of healthy tissue surrounding it to minimize the risk of recurrence. It’s often used for smaller tumors that haven’t spread.
- Radical resection: This more extensive surgery removes the tumor, a wider margin of surrounding tissue, and potentially nearby lymph nodes. It’s often necessary for larger tumors or those that have spread to nearby areas.
- Minimally invasive surgery: In some cases, laparoscopic or robotic surgery may be an option. These techniques use smaller incisions and specialized instruments, which can lead to less pain, scarring, and faster recovery times.
Recovery time after surgery can vary depending on the type and extent of the procedure. You’ll likely need to stay in the hospital for a few days to a week. Your health care team will provide you with specific instructions on pain management, wound care, and activity restrictions. It’s important to follow their guidance carefully to ensure a smooth recovery.
If you’re a woman of childbearing age and the tumor is in your uterus, you may want to discuss fertility preservation options with your doctor before surgery. Depending on the location and extent of surgery, reconstructive procedures may be available to restore function or appearance of where the surgery is needed.
Radiation therapy
Radiation therapy is a common treatment approach for leiomyosarcoma by a radiation oncologist. It is used alone or in combination with surgery and/or chemotherapy. It uses high-energy rays (like X-rays) to damage and destroy cancer cells, hindering their ability to grow and divide. It may also be used before or after surgery or for symptom relief (palliative radiation) to help alleviate pain, pressure, or bleeding caused by the tumor.
Types of radiation therapy includes:
- External beam radiation therapy (EBRT): This is the most common type, where radiation is delivered from a machine outside the body, targeting the tumor precisely.
- Internal radiation therapy (brachytherapy): This involves placing radioactive material inside or near the tumor, delivering a high dose of radiation directly to the cancer cells while sparing surrounding healthy tissues.
- 3D conformal radiation therapy (3D-CRT): Shapes the radiation beams to match the tumor’s shape, improving accuracy.
- Intensity-modulated radiation therapy (IMRT): Further refines 3D-CRT by adjusting the intensity of the radiation beams, delivering higher doses to the tumor while minimizing exposure to healthy tissues.
Chemotherapy
Chemotherapy is an important treatment option for leiomyosarcoma, often used alongside surgery and/or radiation therapy. Chemotherapy uses powerful drugs to kill cancer cells or stop them from growing and dividing. Unlike surgery or radiation, which target specific areas, chemotherapy works systemically, meaning the drugs travel throughout the body to reach cancer cells that may have spread beyond the original tumor. It may also be used before or after surgery or for symptom relief (palliative chemotherapy) to help alleviate pain or pressure caused by the tumor.
There are many different chemotherapy drugs, and they are often used in combinations tailored to the individual patient and the characteristics of the cancer. The specific drugs and dosages will be determined by your oncologist.
Chemotherapy can be administered in various ways:
- Intravenously (IV): The drugs are injected directly into a vein, usually in your arm or hand.
- Orally: Chemotherapy pills or capsules are taken by mouth.
- Other methods: Less commonly, it might be given via injection into a muscle, under the skin, or directly into the abdomen (intraperitoneal chemotherapy).
Chemotherapy can cause side effects because the drugs can also affect healthy cells. Your health care team will monitor you closely for side effects and offer medications and strategies to manage them. Chemotherapy can affect fertility, so it’s important to discuss this with your doctor before starting treatment if you are concerned.
Targeted therapy
Targeted therapy for leiomyosarcoma (LMS) aims to attack specific vulnerabilities within the cancer cells, rather than broadly targeting all rapidly dividing cells like chemotherapy does. This can potentially lead to more effective treatment with fewer side effects. However, it’s important to understand that targeted therapy options for LMS are currently limited compared to some other cancers, and they’re typically considered when other treatments (like surgery and chemotherapy) are no longer effective or are not an option.
Immunotherapy
Immunotherapy for leiomyosarcoma (LMS) aims to harness the power of the body’s own immune system to fight the cancer. Unlike chemotherapy and targeted therapies, which directly attack cancer cells, immunotherapy works by helping the immune system recognize and destroy cancer cells.
Leiomyosarcoma FAQs
Leiomyosarcoma is a complex cancer that is often hard to spot early on. Having an understanding about the disease and what to look for may help in early detection. These FAQs are designed to provide you with answers, and give you background information that will help aid in your conversations with your AHN Care Team.
Why is leiomyosarcoma so deadly?
Leiomyosarcoma (LMS) is a challenging cancer because it can occur in parts of the body that don’t show obvious symptoms, like deep in the abdomen. Because of this, it is often found late and after it has spread through the bloodstream to other parts of the body like the lungs or liver. Treatment of LMS is varied, depending on the patient and the extent of the disease. Some LMS cells may not respond to chemotherapy or radiation therapy, making it harder to control the cancer. It’s important to remember that not everyone with LMS will have a poor outcome. Early detection and treatment can significantly improve the chances of success.
What is the survival rate for leiomyosarcoma?
Survival rates for LMS can vary widely depending on several factors, including:
- Stage: How far the cancer has spread when it’s diagnosed.
- Grade: How aggressive the cancer cells look under a microscope.
- Location: Where the cancer started in the body.
- Treatment: The type of treatment you receive and how well you respond.
- Overall health: Your general health and well-being.
Because of these factors, it’s hard to give a single, precise survival rate. Survival rates are often expressed as "five-year survival rates," which means the percentage of people who are still alive five years after their diagnosis.
In general:
- Localized LMS (hasn’t spread): The five-year survival rate can be relatively high, often around 60 – 80%.
- Advanced LMS (has spread): The five-year survival rate is lower, typically around 20 – 40%.
What are the early symptoms of leiomyosarcoma?
Early symptoms of LMS can be vague and depend on where the cancer starts. Some people might not have any symptoms at all in the early stages. When symptoms do occur, they might include:
- A new lump or swelling: Often painless, usually in the arms, legs, or abdomen.
- Abdominal pain or pressure: A general feeling of discomfort in the abdomen.
- Changes in bowel or bladder habits: Constipation, diarrhea, or frequent urination.
- Unusual vaginal bleeding: In women with uterine LMS.
What are the odds of getting leiomyosarcoma?
Leiomyosarcoma (LMS) is a rare cancer. It accounts for only about 1% of all adult cancers. The lifetime risk of developing LMS is estimated to be less than 1 in 100,000 people.
Is leiomyosarcoma cancer curable?
Whether LMS is curable depends on several factors, including the stage and grade of the cancer, where it’s located, and how well it responds to treatment.
- Early-stage LMS: If the cancer is found early and hasn’t spread, surgery to remove the tumor can sometimes be curative.
- Advanced LMS: If the cancer has spread to other parts of the body, a cure may not be possible. However, treatment can often control the cancer, slow its growth, and relieve symptoms, allowing you to live longer and have a better quality of life.
Even if a cure isn’t possible, it’s important to remember that there are many treatment options available, and new treatments are being developed all the time.
Contact us
To make an appointment you can:
- Call our scheduler: (412) 359-8614
- Schedule online
- Use MyChart and schedule with Dr. Lisa Ercolano to make an appointment
Please bring all relevant imaging on CD or through PowerShare with correlating reports for review by our team to your initial appointment.