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Causes, Risk Factors, and Prevention of Uterine Sarcoma
Learn more about the risk factors for uterine sarcoma. The cause of most uterine sarcomas is not completely understood, but knowing whether you have risk factors might be helpful.
What causes uterine sarcoma?
Researchers have found a few risk factors that make a person more likely to develop uterine sarcoma. But having a risk factor doesn’t mean you’ll get the disease. Most people with risk factors don’t develop uterine sarcoma.
Researchers have made great progress in understanding how certain gene changes (mutations) can cause normal cells to become cancerous. For example, some genes contain instructions for controlling when our cells grow and divide. Changes in these genes can lead to cancer growth.
For example:
- Changes in genes such as RB1, TP53, and PTEN have been found in uterine leiomyosarcomas. These genes are tumor suppressor genes and, when mutated, can cause uncontrolled cell growth.
- Low‐grade endometrial stromal sarcoma (ESS) is often linked with abnormal gene changes called fusions with the JAZF1, PHF1, or EPC1 genes.
- High‐grade ESS has been linked with the abnormal YWHAE‐NUTM2 gene or changes in the BCOR gene.
To learn more, see Genes and Cancer.
Risk factors for uterine sarcoma
A risk factor is anything that affects your chance of getting a disease such as cancer. Different cancers have different risk factors. Some risk factors, like smoking, can be changed. Others, like your age or family history, can’t be changed.
Having a risk factor, or even several, does not mean that a person will get the disease. And many people who get the disease might have few or no known risk factors.
Researchers have found a few risk factors that increase the chance of having uterine sarcoma.
Uterine sarcomas are rare, but they are most common in females over the age of 40. Leiomyosarcomas are more common in Black females compared with White females.
Most uterine sarcomas have no clear cause. In rare cases, though, inherited gene changes can cause uterine sarcomas in several members of the same family.
Hereditary retinoblastoma
People with a uterus who have changes in the RB1 gene, which causes retinoblastoma in childhood, have an increased risk of soft tissue sarcomas, including uterine sarcomas, as they age.
Visit Hereditary Retinoblastoma (RB1) for more information.
Hereditary leiomyomatosis and renal cell carcinoma syndrome (HLRCC)
This rare family cancer syndrome has been linked to an increased risk of uterine sarcomas, which might be diagnosed at a younger age than is typical.
Environmental risk factors are things in your surroundings that increase your risk of getting a disease like cancer. Certain medications and radiation are environmental risk factors for uterine sarcoma.
Tamoxifen
Although the risk is small, taking tamoxifen to prevent or treat breast cancer increases the risk of developing uterine cancers. Uterine sarcomas often show up 2 to 5 years after starting tamoxifen. People taking tamoxifen should have age-appropriate gynecologic screening and care. If you have abnormal bleeding from the uterus or other concerning symptoms, visit your doctor so a cause can be found and treated, if needed.
Pelvic radiation therapy
Radiation to the pelvis can increase the risk for developing uterine sarcoma. A uterine sarcoma might occur after someone has had radiation to treat cervical or rectal cancer. These cancers usually are diagnosed 5 to 20 years after the radiation treatment.
Can uterine sarcoma be prevented?
Most cases of uterine sarcoma cannot be prevented.
Pelvic radiation
Although pelvic radiation increases the risk of developing a uterine sarcoma, the benefit of pelvic radiation in treating other cancers outweighs the risk of developing a rare cancer such as uterine sarcoma many years later.
Tamoxifen
The risk of developing uterine sarcomas while taking tamoxifen is small, and there are no specific things you can do to prevent one if you're taking it. People taking tamoxifen should have age-appropriate gynecologic screening and care. If you have abnormal bleeding from the uterus or other concerning symptoms, visit your doctor so a cause can be found and treated, if needed.
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- References
Developed by the 黑料大湿Posts Cancer Society medical and editorial content team with medical review and contribution by the 黑料大湿Posts Society of Clinical Oncology (ASCO).
Boggess JF, Kilgore JE, Tran AQ. Chapter 85: Uterine Cancer. In: Niederhuber JE, Armitage JO, Dorshow JH, Kastan MB, Tepper JE, eds. Abeloff’s Clinical Oncology. 6th ed. Philadelphia, Pa. Elsevier: 2020.
Committee Opinion No. 601: Tamoxifen and uterine cancer. Obstet Gynecol. 2014;123(6):1394-1397.
Hoffman M, Roberts WS, Cavanagh D. Second pelvic malignancies following radiation therapy for cervical cancer. Obstet Gynecol Surv. 1985;40(10):611-617.
Lee PJ, Yoo NS, Hagemann IS, et al. Spectrum of mutations in leiomyosarcomas identified by clinical targeted next-generation sequencing. Exp Mol Pathol. 2017;102(1):156-161.
McPartland C, Salib A, Banks J, et al. Risk of Secondary Malignancies After Pelvic Radiation: A Population-based Analysis. Eur Urol Open Sci. 2024;63:52-61. Published 2024 Mar 23. doi:10.1016/j.euros.2024.02.013
Memarzadeh S, Berek J. Uterine sarcoma: Classification, epidemiology, clinical mainfestations, and diagnosis. UpToDate. 2026. Accessed at https://www.uptodate.com/contents/uterine-sarcoma-classification-epidemiology-clinical-manifestations-and-diagnosis on February 17, 2026.
Yu G, Wei R, Li S, et al. Risk and prognosis of second corpus uteri cancer after radiation therapy for pelvic cancer: A population-based analysis. Front Oncol. 2022;12:957608. Published 2022 Sep 29.
Last Revised: June 9, 2026
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