Definition

Leiomyosarcoma (LMS) is a rare form of cancer that originates in smooth muscle. Smooth muscle is found in hollow organs such as the intestines, stomach, bladder, blood vessels, and the uterus in women. This muscle type performs various functions in the body, working without conscious awareness.

Leiomyosarcoma is a malignant cancer, meaning the cells can grow uncontrollably and spread to nearby organs. It is classified as a soft tissue sarcoma, a type of cancer that affects tissues that support and connect other body structures.

Leiomyosarcoma differs from leiomyoma, which is a benign (non-cancerous) tumor that forms in muscle tissue. While leiomyomas can be bothersome, they do not spread to other areas of the body and are also known as uterine myomas.

Leiomyosarcomas most commonly occur in the abdominal organs or the uterus. They account for approximately 10-20% of soft tissue sarcomas. This type of cancer is more frequently found in adults than in children.

There are three main types of leiomyosarcoma:

• Somatic Soft Tissue Leiomyosarcoma

The most common type, this cancer affects the connective tissue in the body.

• Skin and Fat Leiomyosarcoma

A rare form that affects the piloerector muscle, found in the skin and eyes.

• Blood Vessel Leiomyosarcoma

The rarest type, originating in the large blood vessels of the body.

Causes

The exact cause of leiomyosarcoma remains unclear. It may develop due to mutated genes, either inherited from parents or caused by changes in the body’s own genes. Cells normally follow a life cycle where they eventually die, but when they mutate, they can grow uncontrollably.

Certain genetic disorders are believed to be linked to the development of leiomyosarcoma, including:

• Hereditary Retinoblastoma

The most common eye cancer in children, this condition begins in the retina and is inherited from parents.

• Li-Fraumeni Syndrome

A rare genetic disorder that increases the risk of cancer in individuals and their families.

• Neurofibromatosis Type 1

A disease causing tumors to grow along nerves in the skin, brain, and other body parts, often accompanied by skin discoloration.

• Tuberous Sclerosis

A rare genetic condition that causes benign tumors to form in organs like the brain, heart, kidneys, lungs, eyes, and skin.

• Werner Syndrome

A genetic disorder that accelerates aging from adolescence or young adulthood, increasing the risk of cancer.

Risk Factor

Several factors have been identified that can increase the likelihood of developing leiomyosarcoma, including:

• Exposure to radiation therapy.

• Contact with certain chemicals.

• Tamoxifen drug therapy (commonly used in breast cancer treatment).

Leiomyosarcoma is not considered to be inherited in individuals who do not have specific genetic conditions. While it can affect both men and women, women are more commonly diagnosed with the condition. Moreover, while this cancer can develop at any age, it is particularly prevalent in individuals over the age of 50.

Symptoms

The symptoms associated with leiomyosarcoma can vary based on the tumor’s size and location. In some cases, individuals with leiomyosarcoma may not experience any symptoms during the early stages of the disease. As the tumor increases in size over time, symptoms may become apparent, which can include:

• Pain

• Fatigue

• Fever

• Abdominal bloating

• Unexplained weight loss

• Nausea and vomiting

• Urinary issues

• Abnormal vaginal bleeding outside the menstrual cycle

• A noticeable lump or swelling under the skin that enlarges over time

Diagnosis

The tests and procedures required to diagnose leiomyosarcoma are influenced by where the tumor is located. Your healthcare provider will inquire about your symptoms, including their duration, and will examine your medical and family history to gather more insight into your health condition. A complete physical test will also be conducted, particularly if the tumor is visible.

Additional diagnostic tests to confirm the diagnosis of cancer may include:

• Imaging Tests

If you display symptoms of leiomyosarcoma, your doctor may utilize radiological procedures, such as MRI, CT scans, angiography (to examine blood vessels), and PET scans (positron emission tomography), to determine the tumor's size and location. These tests will also help in detecting whether the cancer has spread to other areas of the body.

• Biopsy

A biopsy is a process where a sample of the suspected cancerous tissue is extracted and analyzed in a laboratory. This test helps to confirm the specific type of tumor.

In some cases, LMS may be misidentified as another type of tumor or health condition based on radiological tests. A biopsy will definitively confirm if the tumor is indeed LMS. Accurate diagnosis is vital to ensure that the most appropriate treatment is provided.

Management

Treatment plans for cancer will be customized for each individual, taking into account factors such as tumor size, location, and whether cancer has spread to other areas of the body. Your overall health will also be a key consideration when determining the best approach to treatment.

Generally, treatment for leiomyosarcoma may involve the following:

• Surgery

Surgery is considered the most effective treatment for leiomyosarcoma. The main objective of surgery is to remove all of the cancerous tissue, especially if the tumor is small and localized. However, this might not always be feasible if the cancer is large or has affected surrounding organs.

In these cases, the surgeon will aim to remove as much of the tumor as possible. If the entire tumor is successfully excised, there is a significant chance of curing the leiomyosarcoma. However, if some cancer cells remain, the risk of the cancer returning in the same location or spreading to other areas of the body is higher.

• Radiation Therapy

Radiation therapy involves the use of high-energy radiation, such as x-rays or protons, to eliminate cancer cells. It can be used following surgery to destroy any remaining cancerous cells or to prevent the cancer from reoccurring in the future.

• Chemotherapy

Chemotherapy is a treatment that utilizes potent chemicals to target and destroy cancer cells. If the leiomyosarcoma tumor is large or if the cancer has spread to other parts of the body, chemotherapy may be used alongside surgery as part of the treatment regimen.

• Targeted Drug Therapy

Targeted drug therapy aims to focus on specific abnormalities present within cancer cells. By targeting and inhibiting these abnormalities, the therapy can induce cancer cell death. This approach may be considered for advanced leiomyosarcoma. For more personalized advice and to discuss treatment options, you should consult your doctor.

Complications

Leiomyosarcoma is an aggressive form of cancer due to its rapid growth, which can lead to potentially life-threatening complications. The life expectancy of patients diagnosed with leiomyosarcoma depends on several factors, including:

• The location of the tumor in the body.

• Whether the cancer has spread to other areas.

• The extent of tumor tissue removal during surgery.

If leiomyosarcoma is detected early, and the tumor can be successfully surgically removed, the prognosis is generally favorable, with a chance for complete recovery. However, if the tumor is large or has metastasized to other parts of the body, treatment becomes more challenging.

Prevention

Currently, no known method exists to prevent the development of leiomyosarcoma. However, minimizing exposure to risk factors can reduce the likelihood of developing the condition.

When to See a Doctor?

If you experience symptoms such as a deep lump under the skin or a recurrence of the tumor after surgical removal, it is important to consult a healthcare provider promptly.

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