Uterine Sarcoma

Overview

What is uterine sarcoma?

Uterine sarcoma is a type of uterine cancer that typically forms in the muscle layer of your uterus (myometrium). Most uterine cancers form in the lining of your uterus (endometrium) and are called endometrial cancers or carcinomas. Sarcomas, on the other hand, are much less common. There are also other rare types of sarcoma that start in the supporting cells of the uterine lining.

Is uterine sarcoma aggressive?

Uterine sarcoma typically grows faster and spreads more quickly than more common endometrial cancers. Still, not all uterine sarcomas are equally aggressive. Your provider will consider where the sarcoma is located and what type it is to determine how aggressive it is.

Your provider can also stage your cancer to see if it has spread.

What is cancer staging?

One of the biggest concerns about a cancer diagnosis is whether it's metastasized (spread) beyond its original location. To determine cancer spread, your provider assigns a number (I through IV) to the diagnosis. The higher the number, the more cancer has spread throughout your body. This classification system is called "staging." Your provider uses this information to plan for treatment.

What are the stages of uterine sarcoma?

The stages of uterine sarcoma are:

  • Stage I: Cancer is in your uterus only.
  • Stage II: Cancer has spread in your pelvis beyond your uterus.
  • Stage III: Cancer has spread to areas in the abdomen outside the pelvis, including lymph nodes.
  • Stage IV: Cancer has spread to distant areas outside the abdomen or is involving the bladder or rectum.

Who gets uterine sarcomas?

Most people diagnosed with uterine sarcomas are over 40, but people as young as 20 have received a uterine sarcoma diagnosis. The average age of diagnosis is approximately 60 years old.

Who is at risk for having uterine sarcoma?

Certain factors may put you at greater risk of having uterine sarcoma.

  • Pelvic radiation. Radiation treatment in your pelvic area may increase your risk of developing uterine sarcoma. Sarcomas rarely form after pelvic radiation, but when they do, they usually appear five to 25 years after radiation.
  • Tamoxifen. Long-term use of tamoxifen to treat breast cancer (five or more years) increases your risk of developing uterine sarcoma.
  • Genetics. Possessing the gene that causes an eye cancer called retinoblastoma increases your risk of developing some types of uterine sarcoma.

People who are Black are twice as likely as people who are white to develop uterine sarcomas than people who are white. Researchers aren't sure why race is a risk factor when it comes to uterine sarcoma.

How common is uterine sarcoma?

Uterine sarcoma is rare, making up only 3% to 7% of all uterine cancers.

Symptoms and Causes

What are the signs and symptoms of uterine sarcoma?

The main symptoms of uterine sarcoma are similar to endometrial cancer and non-cancerous growths, such as fibroids. It's important to see your provider if you notice any of these signs:

You may not notice symptoms until the uterine sarcoma has progressed to a more advanced stage. In rare instances, people with uterine sarcoma are asymptomatic (don't notice symptoms).

What causes uterine sarcomas?

Uterine sarcoma develops when malignant cells form, divide and spread throughout the body. These cells form primarily in the muscular wall of your uterus (myometrium).

Scientists don't know what causes uterine sarcomas. Research to better understand how to diagnose, treat and prevent them is ongoing.

Diagnosis and Tests

How is uterine sarcoma diagnosed?

If your provider thinks you might have uterine sarcoma, they will perform a physical examination and take your medical history. Your provider will also conduct a pelvic examination of your vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. To examine these organs, your provider inserts a gloved finger or two into your vagina and rectum to feel for anything unusual. They may also use a speculum to look inside your vagina.

Your doctor may also perform the following procedures:

  • Transvaginal ultrasound: Ultrasound creates images of soft tissue structures, including your reproductive organs. A specialized transducer is inserted in your vaginal canal about 2 to 3 inches to examine your uterus and ovaries. Sometimes uterine sarcoma and fibroids appear similar on an ultrasound.
  • Endometrial biopsy: Your provider removes a tissue sample from the lining of your uterus for examination.

Your provider can confirm a uterine sarcoma by examining its cells underneath a microscope after either a biopsy or a hysterectomy.

You may need additional tests to stage your cancer once you've been diagnosed with uterine sarcoma. Magnetic resonance imaging (MRI), CT scans, PET scans, and chest X-rays can reveal whether your cancer has spread throughout your body.

Your provider may refer you to a gynecologist who specializes in cancer (gynecological oncologist) to help with both diagnosis and treatment for uterine sarcoma.

Management and Treatment

How is uterine sarcoma treated?

Your provider may use one or more of these therapies to treat uterine sarcoma: surgery, radiation therapy, chemotherapy, and hormone therapy.

Surgery

Surgery to remove the cancer is the most commonly used treatment for uterine sarcoma. The surgeon may remove the mass only. Or, they may perform one of these procedures to remove affected tissue and organs, too:

  • Hysterectomy: removal of your uterus and cervix (sometimes called a total hysterectomy). Your provider may use a tool called a laparoscope to remove your uterus using minimally invasive surgery.
  • Total hysterectomy with salpingo-oophorectomy: removal of your uterus and one or both ovaries and one or both fallopian tubes.
  • Radical hysterectomy: removal of your uterus, cervix, both fallopian tubes, and some surrounding tissue including a portion of the vagina.
  • Lymphadenectomy: removal of your lymph nodes to check them for cancer.
  • Laparotomy: an incision is made in your abdomen, which is examined for cancer. In some cases, your provider may remove other organs affected by cancer.

Radiation therapy

Radiation therapy is a form of cancer treatment that uses high-energy X-rays to kill cancer cells while minimizing damage to healthy cells. Radiation therapy can either be internal (placed in your body) or external (delivered by a machine outside your body). You may receive both internal and external radiation treatment for uterine sarcoma.

Side effects of radiation therapy include:

  • Fatigue (feeling tired).
  • Diarrhea.
  • Nausea and vomiting.
  • Changes to your skin.
  • Irritations in your bladder.
  • Edema (swelling) in your legs.

Chemotherapy

Chemotherapy uses medications to kill or slow the growth of rapidly multiplying cancer cells. These medications are often given intravenously (through a needle into a blood vessel) and can have major side effects. You may receive radiation therapy and chemotherapy together.

Side effects of chemotherapy include:

  • Nausea and vomiting.
  • Hair loss.
  • Loss of appetite.
  • Low blood count.
  • Fatigue.

Hormone therapy

Hormones are substances your body produces to regulate certain functions. Examples include testosterone and estrogen. In some cases, hormones may help cancer to grow.

Hormone therapy stops cancer growth by blocking the action of the hormones. Hormones, such as progestins, gonadotropin-releasing hormone agonists and aromatase inhibitors, are used in treating uterine sarcoma.

Prevention

How can I prevent uterine sarcoma?

We don't know how to prevent uterine sarcoma yet, but we know about certain risk factors, such as receiving pelvic area radiation and taking tamoxifen for breast cancer. Treatment benefits often outweigh the risks associated with developing uterine sarcoma, especially since it's so rare.

Speak to your provider about the risks associated with any cancer treatment, and weigh the pros and cons of the treatment options available.

Outlook / Prognosis

What is the prognosis (outlook) following treatment for uterine sarcoma?

Uterine sarcoma is more concerning and harder to treat than other uterine cancers. Still, the prognosis for uterine sarcoma depends on the type of tumor you have, the grade of cancer (how aggressive the cancer cells look under the microscope) and how much it's spread throughout your body. Speak to your provider about your prognosis based on your unique case.

After treatment, plan to see your provider regularly for follow-up. Being diagnosed with uterine sarcoma may place you at greater risk for certain other types of cancer.

Is uterine sarcoma cancer curable?

Uterine sarcoma is curable, especially if it's low-grade (mildly abnormal cells) and it hasn't spread beyond your uterus. In some cases, additional treatment, like chemotherapy and radiation, may be needed to destroy the cancer cells completely.

How long does a person live with uterine sarcoma?

There's no way to know for sure how long a person with uterine sarcoma will live. Your outcomes will depend on the type of sarcoma you have, how much it's spread, your age, your health and how your body is responding to treatment, among other factors.

According to the National Cancer Institute, the survival rate for uterine sarcomas, five years post-diagnosis, ranges from 41% to 95%, depending on the type of sarcoma and how much it's spread.

Living With

What questions should I ask my doctor?

  • How often will I need follow-up tests and imaging procedures?
  • What will follow-up tests and exams check for?
  • What symptoms signal that my cancer has returned?
  • What everyday habits would you recommend to stay healthy?
  • How will my care team stay in contact and up-to-date about my care plan for treatment?
  • How will the type of uterine sarcoma I have affect my treatment options and prognosis?

A note from Cleveland Clinic

If you are diagnosed with cancer, it's important to realize that you're not alone. Ask your provider for information about support groups for individuals who've been diagnosed with uterine cancer who are receiving treatment. You can also contact your local chapter of the American Cancer Society for more information. In addition, your doctor can refer you to a social worker or a mental health professional to help you deal with the emotional aspects of receiving a cancer diagnosis.

Last reviewed by a Cleveland Clinic medical professional on 01/31/2022.

References

  • American Cancer Society. Multiple pages reviewed for this article. (https://www.cancer.org/cancer/uterine-sarcoma.html) Accessed 2/15/2022.
  • Bužinskienė D, Mikėnas S, Drąsutienė G, Mongirdas M. Uterine sarcoma: a clinical case and a literature review. (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6591694/) Acta Med Litu. 2018;25(4):206-218. Accessed 2/15/2022.
  • Nordal RR, Thoresen SO. Uterine sarcomas in Norway 1956-1992: incidence, survival and mortality. (https://pubmed.ncbi.nlm.nih.gov/9291814/) Eur J Cancer. (https://pubmed.ncbi.nlm.nih.gov/9291814/) (https://pubmed.ncbi.nlm.nih.gov/9291814/) 1997;33(6):907-911. Accessed 2/15/2022.
  • Ricci S, Stone RL, Fader AN. Uterine leiomyosarcoma: Epidemiology, contemporary treatment strategies and the impact of uterine morcellation. (https://pubmed.ncbi.nlm.nih.gov/28209496/) Gynecol Oncol. 2017;145(1):208-216. Accessed 2/15/2022.
  • Sherman ME, Devesa SS. Analysis of racial differences in incidence, survival, and mortality for malignant tumors of the uterine corpus. (https://pubmed.ncbi.nlm.nih.gov/12833470/) Cancer. 2003;98(1):176-186. Accessed 2/15/2022.
  • Tropé CG, Abeler VM, Kristensen GB. Diagnosis and treatment of sarcoma of the uterus. A review. (https://pubmed.ncbi.nlm.nih.gov/22793037/) Acta Oncol. 2012;51(6):694-705. Accessed 2/15/2022.

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