Discover the rare yet aggressive uterine sarcoma, its symptoms, causes, and treatments.

A cancer called uterine sarcoma develops in the uterus’s (the womb’s) muscle or other tissues. It is different from endometrial cancer, which is much more common and starts in the lining of the uterus. Uterine sarcoma tends to grow faster and spread more quickly than endometrial cancer, making it more difficult to treat and cure.

In this blog post, we will cover the following topics:

• What are the symptoms of uterine sarcoma?
• What are the causes of uterine sarcoma?
• What are the risk factors for uterine sarcoma?
• How is uterine sarcoma diagnosed?
• What are the treatment options for uterine sarcoma?
• What are the emerging treatment options for uterine sarcoma?
• What are the future directions of uterine sarcoma research?

We hope that by reading this post, you will gain a better understanding of this rare and aggressive cancer and feel more empowered to make informed decisions about your care.

Uterine Sarcoma: An Introduction

Uterine sarcoma is a term that refers to different types of cancer that originate in the muscle or other tissues of the uterus. There are three main types of uterine sarcoma:

• Leiomyosarcoma, which develops in the smooth muscle cells of the uterus. This is the most common type of uterine sarcoma, accounting for about 60% of cases.
• Endometrial stromal sarcoma, which develops in the connective tissue cells that support the lining of the uterus. This is a less common type of uterine sarcoma, accounting for about 15% of cases.
• Undifferentiated sarcoma, which develops in either the muscle or the lining of the uterus and has no clear features that distinguish it from other types of cancer. This is the rarest and most aggressive type of uterine sarcoma, accounting for about 5% of cases.

Uterine sarcoma can occur at any age, but it is more common in women who are in their 40s through their 60s.The typical diagnosis age is in the range of 60 years old. Uterine sarcoma affects Black women twice as often as white women.

Although the specific aetiology of uterine sarcoma is unknown, some variables may make people more likely to get the condition. These include exposure to pelvic radiation, long-term use of tamoxifen (a drug used to treat breast cancer), and having a genetic condition called hereditary retinoblastoma (a rare eye cancer that occurs in children).

What are the symptoms of uterine sarcoma?

Uterine sarcoma can cause various symptoms, depending on the size and location of the tumor. Some of the most common symptoms include:

• Abnormal vaginal bleeding after menopause or throughout periods
• Pelvic or abdominal pain or pressure
• A feeling of fullness or bloating in the abdomen
• A mass or lump in the pelvis or vagina
• Frequent urination or difficulty urinating
• Vaginal discharge that does not improve with medication
• Constipation or difficulty passing stool

These symptoms can also be caused by other conditions, such as fibroids (non-cancerous growths in the uterus), endometriosis (a disorder where tissue similar to the lining of the uterus grows outside the uterus), or infections. Therefore, it is important to see your doctor if you experience any unusual or persistent symptoms that affect your reproductive organs.

What are the causes of uterine sarcoma?

The exact cause of uterine sarcoma is not known, but researchers believe that it may result from changes or mutations in the DNA of certain cells in the uterus. These changes can make the cells grow and divide abnormally, forming a tumor. Some factors may increase the likelihood of these changes occurring, such as:

• Exposure to radiation: Radiation therapy used to treat other cancers in the pelvic area can damage the DNA of healthy cells and increase the risk of developing uterine sarcoma later in life. The risk is higher if radiation is given at a young age or at high doses.
• Use of tamoxifen: Tamoxifen is a drug that blocks estrogen receptors on breast cancer cells and prevents them from growing. However, tamoxifen can also act as an estrogen-like substance on other tissues, such as the uterus. Long-term use of tamoxifen (more than five years) can increase the risk of developing uterine sarcoma, especially leiomyosarcoma.
• Genetic factors: Some people may inherit a gene that increases the risk of developing certain types of cancer, including uterine sarcoma. One example is the RB1 gene, which normally helps control cell growth and prevent tumors. People who have a mutation in this gene have a condition called hereditary retinoblastoma, which causes eye cancer in children. They also have a higher risk of developing other cancers, such as osteosarcoma (bone cancer), melanoma (skin cancer), and uterine sarcoma.

What are the uterine sarcoma risk factors?

Anything that increases your likelihood of contracting an illness is a risk factor. Having a risk factor does not mean that you will get the disease, and not having a risk factor does not mean that you will not get the disease. Some risk factors for uterine sarcoma are:

• Age: Uterine sarcoma is more common in women who are in their 40s through their 60s. The typical diagnosis age is in the range of 60 years old.
• Race: Uterine sarcoma affects Black women twice as often as white women.
• Radiation exposure: Women who have had radiation therapy to the pelvic area for other cancers have a higher risk of developing uterine sarcoma later in life.
• Tamoxifen use: Women who have taken tamoxifen for more than five years to treat or prevent breast cancer have a higher risk of developing uterine sarcoma, especially leiomyosarcoma.
• Genetic factors: Women who have a mutation in the RB1 gene or other genes that increase the risk of cancer have a higher risk of developing uterine sarcoma.

How is uterine sarcoma diagnosed?

Uterine sarcoma can be hard to diagnose because it can cause symptoms similar to other conditions, such as fibroids or endometrial cancer. There is no specific screening test for uterine sarcoma, and a Pap smear (a test that checks for cervical cancer) does not detect it. Therefore, it is important to see your doctor if you have any unusual or persistent symptoms that affect your reproductive organs.

To diagnose uterine sarcoma, your doctor may perform one or more of the following tests:

• Physical exam: Your doctor will examine your pelvis and abdomen to check for any masses or lumps. They will also ask you about your medical history, family history, and symptoms.
• Pelvic ultrasound: This is a test that uses sound waves to create images of your uterus and other organs in your pelvis. It can help detect any abnormal growths or changes in the shape or size of your uterus.
• Hysteroscopy: This is a procedure that uses a thin, lighted tube (called a hysteroscope) to look inside your uterus through your vagina and cervix. It can help detect any abnormal tissue or bleeding in your uterus.
• Endometrial biopsy: This is a procedure that uses a thin, hollow needle or a small instrument to remove a sample of tissue from the lining of your uterus. The tissue is then examined under a microscope to look for any signs of cancer.
• Dilation and curettage (D&C): This is a procedure that uses a thin instrument (called a curette) to scrape away tissue from the lining and wall of your uterus. The tissue is then examined under a microscope to look for any signs of cancer.
• Laparoscopy: This is a procedure that uses a thin, lighted tube (called a laparoscope) to look inside your abdomen through small incisions in your skin. It can help detect any signs of cancer spread to other organs or tissues in your abdomen.
• CT scan: This is a test that uses x-rays to create detailed images of your abdomen and pelvis. It can help detect any signs of cancer spread to other organs or tissues in your abdomen or pelvis.
• MRI scan: This is a test that uses magnetic fields and radio waves to create detailed images of your abdomen and pelvis. It can help detect any signs of cancer spread to other organs or tissues in your abdomen or pelvis.
• PET scan: This is a test that uses a radioactive substance (called a tracer) to show how cells are working in different parts of your body. It can help detect any signs of cancer activity or spread in your body.

The only way to confirm the diagnosis of uterine sarcoma is by examining the tissue under a microscope by a pathologist (a doctor who specializes in diagnosing diseases by looking at cells and tissues). The pathologist will also determine the type and grade (how abnormal the cells look) of the uterine sarcoma.

What are the treatment options for uterine sarcoma? 

The treatment options for uterine sarcoma depend on several factors, such as the type, grade, stage (how far the cancer has spread), and location of the tumor, as well as the age, general health, and preferences of the patient. The main types of treatment for uterine sarcoma are:

Surgery

This is the most common and effective treatment for uterine sarcoma. Surgery involves removing the tumor and some surrounding healthy tissue to ensure that no cancer cells are left behind. Depending on the extent of the disease, surgery may include:

• Hysterectomy: This is a surgery that removes the uterus and cervix. It may be done through an incision in the abdomen (abdominal hysterectomy) or through the vagina (vaginal hysterectomy). Sometimes, the ovaries and fallopian tubes are also removed (bilateral salpingo-oophorectomy).
• Radical hysterectomy: This is a surgery that removes the uterus, cervix, ovaries, fallopian tubes, and part of the vagina. It may also remove some lymph nodes in the pelvis (pelvic lymph node dissection) and some tissue around the bladder and rectum (parametrectomy).
• Debulking surgery: This is a surgery that removes as much of the tumor as possible, but not all of it. It may be done when the tumor is too large or too widespread to be removed completely. It may help reduce symptoms and improve the effectiveness of other treatments.
• Metastasectomy: This is a surgery that removes tumors that have spread to other parts of the body, such as the lungs or liver. It may be done when there are only a few metastases and they can be removed safely.

Surgery may cause side effects such as pain, bleeding, infection, scarring, nerve damage, bladder or bowel problems, sexual dysfunction, infertility, and early menopause.

Radiation therapy

This is a treatment that uses high-energy rays or particles to kill cancer cells or stop them from growing. Radiation therapy may be given before or after surgery to shrink the tumor or prevent it from coming back. It may also be given alone or with chemotherapy to treat advanced or recurrent uterine sarcoma. There are two ways to give Radiation Therapy:

• External beam radiation therapy: This is a type of radiation therapy that delivers radiation from a machine outside the body. The radiation is directed at the tumor and some surrounding tissue. It is usually given in daily sessions for several weeks.
• Internal radiation therapy (brachytherapy): This is a type of radiation therapy that delivers radiation from a source inside the body. The source may be a radioactive implant (such as a seed, wire, or pellet) that is placed in or near the tumor, or a radioactive liquid (such as an injection or a drink) that is taken into the body. The radiation stays in the body for a short time and affects only a small area.

Radiation therapy may cause side effects such as skin irritation, fatigue, nausea, vomiting, diarrhea, bladder irritation, vaginal dryness, narrowing or shortening of the vagina (vaginal stenosis), sexual dysfunction, infertility, and early menopause.

Chemotherapy

In this form of treatment, cancer cells are either killed or prevented from proliferating using medications. Chemotherapy may be given before or after surgery to shrink the tumor or prevent it from coming back. It may also be given alone or with radiation therapy to treat advanced or recurrent uterine sarcoma. Chemotherapy may be given in different ways:

• Systemic chemotherapy: This kind of chemotherapy uses the bloodstream to carry medications to cancer cells all over the body. The drugs may be given by mouth (orally) or by injection into a vein (intravenously). Systemic chemotherapy is usually given in cycles of treatment followed by rest periods.
• Regional chemotherapy: This is a type of chemotherapy that delivers drugs directly to a specific area of the body where the cancer is located. The drugs may be given by injection into an artery (intra-arterial) or into the abdominal cavity (intraperitoneal). Regional chemotherapy may help reduce side effects and increase effectiveness.

Chemotherapy may cause side effects such as hair loss, mouth sores, loss of appetite, nausea, vomiting, diarrhea, low blood cell counts, increased risk of infection, bleeding problems, fatigue, nerve damage (neuropathy), kidney damage (nephrotoxicity), heart damage (cardiotoxicity), and early menopause.

Targeted therapy

This is a treatment that uses drugs or other substances to target specific molecules involved in cancer growth and survival. Targeted therapy may work differently from chemotherapy by blocking signals that tell cancer cells to grow and divide or by helping the immune system recognize and destroy cancer cells. Targeted therapy may be given alone or with other treatments to treat advanced or recurrent uterine sarcoma. There are different ways to give Targeted therapy:

• Oral targeted therapy: This is a type of targeted therapy that delivers drugs by mouth (orally) to reach cancer cells throughout the body. The drugs may be taken daily or on a schedule.
• Intravenous targeted therapy: This is a type of targeted therapy that delivers drugs by injection into a vein (intravenously) to reach cancer cells throughout the body. The drugs may be given in cycles of treatment followed by rest periods.

Targeted therapy may cause side effects such as skin rash, diarrhea, nausea, vomiting, high blood pressure, liver problems, bleeding problems, fatigue, and low blood cell counts.

Some examples of targeted therapy drugs that are approved or being tested for uterine sarcoma are:

• Pazopanib (Votrient): This is a drug that blocks several enzymes (called tyrosine kinases) that are involved in blood vessel formation (angiogenesis) and cancer cell growth and survival. It is approved by the Food and Drug Administration (FDA) for the treatment of advanced soft tissue sarcoma, including uterine sarcoma.
• Trabectedin (Yondelis): This is a drug that binds to DNA and interferes with its function, leading to cancer cell death. It is approved by the FDA for the treatment of advanced soft tissue sarcoma, including uterine sarcoma.
• Olaratumab (Lartruvo): This is a drug that is a monoclonal antibody, which is a type of protein that binds to a specific target on cancer cells or other cells. It targets a protein called platelet-derived growth factor receptor alpha (PDGFR-alpha), which is involved in cancer cell growth and survival. It is approved by the FDA for the treatment of advanced soft tissue sarcoma, including uterine sarcoma, in combination with doxorubicin (a chemotherapy drug).
• Mifamurtide (Mepact): This is a drug that is a synthetic version of a molecule found in bacteria. The immune system is prompted to find and target cancer cells as a result. It is approved in Europe for the treatment of osteosarcoma (bone cancer), but it is being tested in clinical trials for other types of sarcoma, including uterine sarcoma.

Future directions of uterine sarcoma research

Uterine sarcoma is a rare and aggressive cancer that poses many challenges and unmet needs for patients and doctors. Therefore, there is a need for more research and innovation to improve the prevention, diagnosis, treatment, and survival of this disease. Some of the future directions of uterine sarcoma research are:

• Prevention and early detection: Research is needed to identify the risk factors and causes of uterine sarcoma and to develop strategies to prevent or reduce them. Research is also needed to develop better screening methods and biomarkers to detect uterine sarcoma at an early stage when it is more treatable and curable.
• Molecular characterization and classification: Research is needed to understand the molecular and genetic features of uterine sarcoma and how they affect its behavior and response to treatment. Research is also needed to develop more accurate and consistent ways to classify uterine sarcoma based on its molecular and genetic features.
• Novel therapies and combinations: Research is needed to discover new drugs or other substances that can target the specific vulnerabilities of uterine sarcoma cells and stop their growth and survival. Research is also needed to test different combinations of existing or new treatments to find the most effective and least toxic ones for each type and stage of uterine sarcoma.
• Clinical trials and personalized medicine: Research is needed to conduct more clinical trials to test the safety and efficacy of new or existing treatments for uterine sarcoma in different settings and populations. Research is also needed to develop personalized medicine approaches that can tailor the treatment to each patient’s individual characteristics, such as tumor type, grade, stage, location, molecular profile, and preferences.
• Quality of life and survivorship: Research is needed to address the physical, emotional, social, and financial challenges that patients with uterine sarcoma face during and after treatment. Research is also needed to improve the quality of life and long-term outcomes of survivors of uterine sarcoma.

Conclusion

Uterine sarcoma is a rare and aggressive cancer that starts in the muscle or other tissues of the uterus. It can cause various symptoms, such as abnormal vaginal bleeding, pelvic pain, or a mass in the pelvis. It can be hard to diagnose because it can mimic other conditions, such as fibroids or endometrial cancer. There is no specific screening test for uterine sarcoma.

The treatment options for uterine sarcoma depend on several factors, such as the type, grade, stage, and location of the tumor, as well as the age, general health, and preferences of the patient. The main types of treatment are surgery, radiation therapy, chemotherapy, and targeted therapy. These treatments may have side effects that can affect the quality of life and fertility of patients.

There are also emerging treatment options for uterine sarcoma that are still in the early stages of development and testing. These include immunotherapy, gene therapy, nanomedicine, among others. They offer hope and promise for the future of uterine sarcoma treatment and research.

FAQ’s

Here are some frequently asked questions about uterine sarcoma: