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What is Uterine Sarcoma?

Summary to be written

The Basics

Diagnosis and Prognosis

The Basics

Uterine sarcoma is a rare type of cancer that originates in the muscle tissue of the uterus. The
uterus is a small, pear-shaped organ in a female's pelvis, and is where a fetus develops and
grows. Uterine sarcoma accounts for about 1% of all female genital malignancies and <10% of
uterine tumors.[see References: 1] Uterine sarcomas include several different subtypes, such as leiomyosarcoma,
endometrial stromal sarcoma, undifferentiated sarcoma, adenosarcoma, and rhabdomyosarcoma.
Among these, leiomyosarcoma and endometrial stromal sarcoma are the most common types of
uterine cancer, accounting for 2-5% of all uterine cancers.[2] Consequently, most of the available
research and clinical data focus on these two subtypes. Uterine sarcomas generally have a
less-favorable prognosis for long-term survival compared to endometrioid adenocarcinoma, the
most common form of uterine cancer. Despite their rarity, evidence suggests that the incidence is
increasing.[3] Because of their rarity and many subtypes, there is no agreed-upon best treatment.

Surgical management remains the cornerstone of treatment for most types of uterine sarcoma, as
these tumors generally show poor response to systemic treatment such as chemotherapy and
immunotherapy.[1, 4] For advanced-stage diagnoses with spread throughout the abdomen,
cytoreductive surgery (CRS) may be a viable treatment option. Even more promising, some
recent studies also indicate that adding hyperthermic intraperitoneal chemotherapy (HIPEC) to
CRS may offer a survival benefit in select patients.[5]

Risk Factors

Due to the rarity of the disease, risk factors are difficult to estimate, but can include:

Increasing age: the majority occur in patients over 40, with a mean age at diagnosis of approximately 60 years
African American women are more frequently diagnosed
History of tamoxifen use >5 years
History of pelvic radiation therapy
Certain inherited syndromes and conditions (renal cell carcinoma, hereditary leiomyomatosis, retinoblastoma)

Signs & Symptoms

Symptoms can be similar to other gynecologic conditions and are often non-specific:

Abnormal uterine bleeding
Postmenopausal bleeding
Abdominal/pelvic pain
Abdominal distension
Urinary symptoms, such as frequent urination, painful urination, difficulty urinating

Evaluation

Physical exam
Imaging studies: ultrasound, PET/CT scans, CT scan, MRI scan
Blood work to include tumor markers
Biopsy (endometrial biopsy, D&C, transabdominal or transvaginal biopsy of mass)

Key Facts

_title_

Uterine sarcomas are very rare and aggressive tumors, and there is limited information on
the most effective treatment options.

_title_
Surgery is the primary treatment, with the best outcomes seen when the entire tumor is
successfully removed. Thus, an experienced surgeon is essential.

_title_

Additional treatments, including radiation, chemotherapy, targeted therapy,
immunotherapy, and hormone therapy, show mixed results. Many clinical trials are
focused on improving and exploring these options further. A medical oncologist and
specialized gynecologic oncologist can help determine which option is best for your case.

_title
While evidence is limited, recent studies suggest that adding HIPEC to cytoreductive
surgery (CRS) may improve progression-free and overall survival in select patients.

Treatment

A standard of care treatment for uterine sarcomas has not yet been established. Surgery is the primary treatment and may be combined with additional therapies, including radiation therapy,chemotherapy, and/or hormone therapy, depending on the stage and their histology (how abnormal the cells look under a microscope) of the disease. The treatment plan is tailored to each patient based on these factors and will most often require multi-disciplinary collaboration, or team-based care between multiple clinicians.

Surgery:

Surgery for uterine sarcoma typically involves at least a total hysterectomy (removal of the uterus and cervix). The extent of the surgery depends on the size of the tumor and how much it has spread within the abdomen. If the disease is confined to the uterus, a total hysterectomy (removal of the uterus and the cervix) is usually sufficient. In many cases, a bilateral salpingo-oophorectomy (BSO) (removal of the both fallopian tubes and ovaries) is also performed during the hysterectomy, particularly in postmenopausal women and those with endometrial stromal sarcomas or undifferentiated sarcomas (a type of histologic subtype).[4]

If the cancer has spread beyond the uterus, more extensive surgery beyond the hysterectomy and BSO may be required to remove all visible tumors within the abdomen. This may involve the removal of pelvic and/or para-aortic lymph nodes if they appear suspicious on imaging or examination during a surgical procedure.[4, 6, 7] Surgery is considered most successful and is associated with the biggest survival increase when all visible tumors are completely removed.[8-12] It is important that patients be treated by an experienced care team to help identify treatment options that take into account several important factors, such as feasibility of removing the tumor, the patient’s ability to undergo extensive surgery, and the timing of the metastases.[13-15] To reduce the risk of recurrence after complete removal of all visible tumors, surgery may be followed by radiation, chemotherapy, and/or hormone therapy.

In many cases, uterine sarcoma (particularly leiomyosarcoma) is diagnosed after surgical procedures to remove the uterus (called a hysterectomy) or uterine fibroids (called a myomectomy) that were initially performed for what was thought to be benign leiomyomas (fibroids).[8, 16] As a result, surgical procedures to remove other organs and tissues (including the uterus, cervix, fallopian tubes, ovaries, and any residual tumors) may be necessary. During the initial surgery, morcellation (the process of grinding fibroids or the uterus into small pieces for easier removal) may have been used. Unfortunately, morcellation can spread tumor cells throughout the abdominal cavity.[17] Therefore, a secondary surgery or re-exploration surgery is often recommended, as many patients develop secondary uterine disease or extrauterine disease.[18, 19] Morcellation should be avoided even in benign cases.

For patients who are not surgical candidates or in whom a complete cytoreduction is not feasible, surgery offers no survival benefit and it may delay the start of systemic treatment like chemotherapy.[20] In such cases, surgery should only be considered for palliative purposes to alleviate significant symptoms such as pain or bleeding.

Chemotherapy:

Uterine sarcomas generally show mixed responses to chemotherapy, with effectiveness varying depending on the specific subtype and molecular changes in the tumor.[24] The effectiveness of adjuvant (postoperative) chemotherapy, especially in early stage disease, remains uncertain.[7, 15] Although no definitive clinical trials have proven that chemotherapy improves progression-free or overall survival, it is commonly recommended for high-risk sage I and II uterine sarcomas, including cases following morcellation, as well as in stage III disease due to the higher risk of recurrence.[25-27] Common chemotherapy drugs that may be considered for use include doxorubicin, docetaxel, gemcitabine, ifosfamide, docarbazine, trabectedin, and eribulin.[28] These may be given as single agents or in combination. In some cases, chemotherapy may be used before surgery to shrink the tumor, making it easier to remove, and then after surgery to prevent delay or recurrence. For patients with advanced, unresectable disease who are not candidates for surgery, chemotherapy is typically the preferred treatment. This may be combined with radiation therapy for palliative purposes.[15]

The role of chemotherapy, including the optimal regimen and drug choice, are still being investigated in clinical trials.[7] It is essential to work with an experienced medical oncologist who collaborates with your surgeon to determine the best treatment approach tailored to your individual case.

Radiation:

Radiation therapy may be administered after surgery (called adjuvant radiation) to reduce the risk of local recurrence.[21] There are two main types of radiation therapy: external beam radiation therapy (EBRT) and vaginal brachytherapy (VBT). EBRT is the more commonly used for uterine sarcoma and targets a broader area, typically including the pelvis and nearby lymph nodes, whereas VBT specifically targets the vaginal cuff. These therapies can be used alone or in combination depending on the individual case. The choice of radiation technique and the area to be treated depend on the extent of the disease, but this remains a topic of ongoing debate. Data on the benefits of adjuvant radiation is limited and mixed, and there is no strong evidence supporting its use in all cases. Available research is primarily focused on leiomyosarcoma or include multiple subtypes of uterine sarcoma, making it difficult to know the benefit in other subtypes like endometrial stromal sarcoma or adenosarcoma.[7] While adjuvant radiation has not been shown to improve survival for early stage disease (stage I & II), it may help prevent or delay local recurrence in some high-risk stage II-IV patients (ie. those with positive surgical margins, potential residual disease, or other aggressive features).[21, 22]

For patients with unresectable tumors who are not surgical candidates, radiation can be used alone or in combination with chemotherapy (chemoradiation). In this scenario, it is intended to manage symptoms such as pain or bleeding caused by tumor growth. However, it is not curative and aims to improve the patient’s quality of life.[23] A specialized radiation oncologist will be consulted to determine the appropriate administration for your specific case.

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC):

When uterine sarcoma spreads throughout the abdominal cavity, it is referred to as peritoneal sarcomatosis and is associated with a poor prognosis, with median survival ranging from 6-15 months.[1] Treatment options are limited in this setting, usually relying on systemic chemotherapy with poor results, indicating the need for alternative treatments.[7, 15] Recent studies have shown that combining extensive cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) significantly improves survival.[5] CRS refers to the aggressive surgical removal or destruction of cancer with the goal to remove all visible disease. It is performed through an incision down the midline of the abdomen and may include removal of parts of or the entirety of organs such as: the spleen, gallbladder, liver, peritoneum (lining of the abdominal cavity), uterus, fallopian tubes/ovaries, and bowel. Following CRS, heated chemotherapy (HIPEC) is circulated directly into the abdominal cavity to kill any remaining microscopic cancer cells.

The largest multi-institutional study on this approach, involving 36 patients with uterine sarcoma, reported a median overall survival of 37 months and a 5-year survival rate of 32%, with a median progression-free survival of 19 months.[29] Another study comparing CRS alone to CRS/HIPEC found that the HIPEC group had longer overall (43.8 vs 35.9 months) and progression-free survival (11.3 vs 5.3 months), although the sample size was small.[30] Other studies suggest that CRS/HIPEC may be beneficial after morcellation or in recurrent cases, offering the potential for long-term survival, especially when complete cytoreduction is achieved in select patients.[31-36] However, since data is still limited, HIPEC is generally recommended only in clinical trials. A skilled surgeon specializing in peritoneal surface malignancies can help determine if CRS/HIPEC is a suitable option for your situation.

Hormone Therapy

Hormone therapy is sometimes used to treat low-grade endometrial stromal sarcoma, though it has not been proven effective for other types of uterine sarcoma.[37] To determine if hormone therapy might be effective, the pathologic evaluation of your tumor will include testing for estrogen (ER) and progesterone receptors (PR). If your tumor is positive for these receptors, hormone therapy may be beneficial. Hormone therapy is usually reserved for cases where the cancer has spread beyond the uterus (advanced stage or metastatic) or has recurred after previous treatment. There are no formal guidelines for the duration of hormone therapy, but it is commonly administered for 5 years.[38] The most frequently used drugs are progestins (such as Provera and Megace), which mimic the action of the hormone progesterone. Some studies have shown that adjuvant therapy with Megace and Provera may improve recurrence rates.[39, 40] Other hormone therapies, including aromatase inhibitors and gonadotropin-releasing hormone (GnRH) analogs, are also being explored.[41, 42] Clinical trials are ongoing to evaluate the effectiveness of other hormonal treatments for endometrial stromal sarcomas. Your medical oncologist and gynecologic oncologist can help determine if hormone therapy is appropriate for you.

Targeted & Immunotherapy

Targeted and immunotherapies are relatively new approaches for treating uterine sarcoma and are usually used for advanced disease, often after chemotherapy has been tried. One such treatment is kinase inhibitors, like pazopanib, which was approved in the United States for metastatic soft tissue sarcomas that have progressed after chemotherapy. A randomized trial showed that pazopanib significantly improved progression-free survival (median: 5 months vs 2 months), although overall survival was similar to the placebo (13 months vs 11 months).[43] Recent studies have also identified a genetic rearrangement in the NTRK genes in some uterine sarcomas. TRK inhibitors, such as larotrectinib and entrectinib, target these abnormal proteins and may offer treatment options for tumors with this specific molecular alteration.[44] In addition, PARP inhibitors can target uterine sarcomas with BRCA mutations, and there is growing interest in combining these with chemotherapy to potentially improve survival.[45] This could be a promising option for the roughly 10% of uterine leiomyosarcoma patients who have a BRCA mutation.[46] Immunotherapy, particularly PD-1 inhibitors like pembrolizumab, may also be used for advanced uterine sarcomas, especially when other treatments have failed and if the tumor has a high tumor mutational burden (TMB).[47] As more targeted treatments become available, sequencing your tumor is increasingly important to identify specific molecular alterations, allowing for more personalized and effective treatment strategies.

Diagnosis and Prognosis

The evolving classification and rarity of uterine sarcomas makes it challenging to compare prognoses. However, in addition to the ability to achieve complete removal of all visible tumor, tumor grade and stage are still the main factors contributing to prognosis. Low-grade (grades 1 & 2) endometrial stromal sarcoma have a 5-year disease free survival of over 90%,while high-grade endometrial stromal sarcomas and undifferentiated uterine sarcomas (grade 3) have a 5-year disease-free survival rate of only 42%.[54] Low-grade endometrial stromal sarcomas also tend to have better overall survival, with 65-76% surviving for 10 years, compared to high-grade cases, which have a median overall survival of just 11-24 months.[60] Leiomyosarcomas have a 5-year survival of 66%, which varies by stage: 75% at stage 1 and decreasing to 29% at stage IV.[61] However, all stages of leiomyosarcoma carry a high risk for relapse (up to 40%), particularly within the first 5 years after treatment.[62, 63] Therefore, close surveillance and follow up, including physical exams, tumor markers, and imaging (such as CT scans) with your gynecologic oncologist are essential.

Recurrence

Uterine sarcomas, regardless of their stage, have a high risk for recurrence, making careful follow up after initial treatment crucial. The recurrence rate after complete resection can be as high as 50-70%.[48, 49] Most recurrences occur in the abdomen or pelvis (40-50%), followed by the lungs (~25%).[50, 51] There have also been reports of recurrences in the spine, hematologic system, and even late recurrences in patients with early-stage disease.[52-54] For patients with localized recurrence or resectable metastatic disease, surgical resection or local ablation techniques are recommended. The best outcomes are seen in patients with long progression-free survival (>12-18 months from initial treatment to recurrence) and limited disease that can be completely resected. Long-term survival is possible following surgical resection.[55-58] Additionally, emerging data suggests that adding HIPEC to cytoreductive surgery may offer survival benefit for recurrent uterine sarcomas.[30]

There is no available data evaluating the role of adjuvant systemic treatment after removal of the metastasis (metastasectomy) or resection of recurrent disease, so the standard approach is surveillance.[59] Hormone therapy can may be considered for low-grade endometrial stromal sarcomas, but it is based on low-quality data from retrospective studies.[37] For patients with unresectable disease recurrence, treatment should focus on palliative care, often including systemic chemotherapy and sometimes radiation.

Research

Clinical Trials

Because uterine sarcomas are so rare, the majority of patients will be recommended to explore clinical trials at some point during their treatment. There are several actively recruiting clinical trials for uterine sarcoma registered online. Below is a sample of a few of the clinical trials listed on www.ClinicalTrials.gov (as of February 2025). Your oncologists (medical, surgical, and gynecologic) can also help you review clinical trial options and recommendations.

Cytoreductive Surgery & HIPEC with Gemcitabine + Chemotherapy with Sacarbazine in Uterine Leiomyosarcoma (NCT04727242)

Status: Recruiting
This phase II single arm study aims to assess the safety, peritoneal disease control, and quality of life in advanced or recurrent leiomyosarcoma patients treated with CRS/HIPEC and adjuvant chemotherapy.
Inclusion Criteria: diagnosis of uterine leiomyosarcoma with recurrence, ability to achieve a complete cytoreduction, life expectancy >3 months, ability to undergo surgery

Letrozole in Uterine Leiomyosarcoma (NCT05649956)

Status: Recruiting
This phase II randomized trial compares the impact of adjuvant letrozole versus observation in newly diagnosed leiomyosarcomas on progression free survival
Inclusion Criteria: stage I or II uterine leiomyosarcoma, estrogen receptor (ER) positive), had a hysterectomy and bilateral salpingo-oophorectomy no more than 12 weeks prior

Study of Lurbinectedin in Combination with Doxorubicin vs Doxorubicin Alone as First-Line Treatment in Participants with Metastatic Leiomyosarcoma (SaLuDo) (NCT06088290)

Status: Recruiting
This phase IIb/III randomized study aims to evaluate whether lurbinectedin + doxorubicin improves progression-free survival compared to doxorubicin alone as first line treatment for metastatic leiomyosarcoma
Inclusion Criteria: diagnosis of metastatic leiomyosarcoma, radiologically measurable disease, no previous systemic therapy for metastatic disease, able to receive systemic treatment

Other Resources

More information on uterine sarcoma

American Cancer Society

cancer.org/uterine-sarcoma

National Cancer Institute

cancer.gov/uterine-sarcoma

Patient Support

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Patient and Caregiver Network

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References

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