Learn about the medical, dental, pharmacy, behavioral, and voluntary benefits your employer may offer.
Uterine sarcoma accounts for less than 1% of gynecologic malignancies and 2% to 5% of all uterine malignancies.[
These three distinct entities are often grouped together as uterine sarcomas; however, each type of tumor is being studied in separate clinical trials.
Carcinosarcoma (the preferred designation by the World Health Organization [WHO]) is also referred to as mixed mesodermal sarcoma or mullerian tumor. Controversy exists about the following issues:
The stromal components of carcinosarcoma are further characterized by homologous elements, such as malignant mesenchymal tissue considered possibly native to the uterus, or heterologous elements, such as striated muscle, cartilage, or bone, which are foreign to the uterus. Carcinosarcoma parallels endometrial cancer in its postmenopausal predominance and in other epidemiological features. Increasingly, the treatment of carcinosarcoma is becoming similar to combined modality approaches for endometrial adenocarcinoma.
Other rare forms of uterine sarcoma also fall under the WHO classification for mesenchymal and mixed tumors of the uterus. These sarcomas include the following:[
For more information, see
Risk Factors
The only documented etiological factor in 10% to 25% of these malignancies is prior pelvic radiation therapy, which is often administered for benign uterine bleeding that began 5 to 25 years earlier. An increased incidence of uterine sarcoma has been associated with tamoxifen in the treatment of breast cancer. Subsequently, increases have also been noted when tamoxifen was given to prevent breast cancer in women at increased risk—a possible result of the estrogenic effect of tamoxifen on the uterus. Because of this increase, patients taking tamoxifen should have follow-up pelvic examinations and should undergo endometrial biopsy if there is any abnormal uterine bleeding.[
Prognosis
The prognosis for women with uterine sarcoma primarily depends on the extent of disease at the time of diagnosis.[
These factors, in addition to the following ones, correlate with a progression-free interval:[
Factors that bear no relationship to the presence or absence of metastases at surgical exploration include:
In one study, women with well-differentiated sarcomatous components or carcinosarcomas had significantly longer progression-free intervals than those with moderately to poorly differentiated sarcomas for the homologous and heterologous types. The recurrence rate was 44% for homologous tumors and 63% for heterologous tumors. The type of heterologous sarcoma had no effect on the progression-free interval.
For women with leiomyosarcomas, some investigators consider tumor size to be the most important prognostic factor. Women with tumors larger than 5.0 cm in maximum diameter have a poor prognosis.[
Surgery alone can be curative if the malignancy is contained within the uterus. The value of pelvic radiation therapy is not established. Current studies consist primarily of phase II chemotherapy trials for patients with advanced disease. Adjuvant chemotherapy following complete resection for patients with stage I or II disease was not found to be effective in a randomized trial.[
References:
The most common histological types of uterine sarcomas include:
The uterine neoplasm classification of the International Society of Gynecologic Pathologists and the World Health Organization uses the term carcinosarcoma for all primary uterine neoplasms containing malignant elements of both epithelial and stromal light microscopic appearances, regardless of whether malignant heterologous elements are present.[
References:
FIGO Staging
The Fédération Internationale de Gynécologie et d'Obstétrique (FIGO) and the American Joint Committee on Cancer have designated staging to define uterine sarcoma; the FIGO system is most commonly used.[
The FIGO staging system has two divisions, one for leiomyosarcoma and endometrial stromal sarcoma, and one for adenosarcoma. Carcinosarcoma is staged using the designated endometrial carcinoma definitions. For more information, see
Stage | Definition |
---|---|
FIGO = Fédération Internationale de Gynécologie et d'Obstétrique. | |
a Adapted from the Fédération Internationale de Gynécologie et d'Obstétrique.[ |
|
I | Tumor limited to uterus. |
–IA | Tumor ≤5 cm. |
–IB | Tumor >5 cm. |
II | Tumor extends beyond the uterus, within the pelvis. |
–IIA | Adnexal involvement. |
–IIB | Involvement of other pelvic tissues. |
III | Tumor invades abdominal tissues (not just protruding into the abdomen). |
–IIIA | One site. |
–IIIB | More than one site. |
–IIIC | Metastasis to pelvic and/or para-aortic lymph nodes. |
IV | |
–IVA | Tumor invades bladder and/or rectum. |
–IVB | Distant metastasis. |
Stage | Definition |
---|---|
FIGO = Fédération Internationale de Gynécologie et d'Obstétrique. | |
a Adapted from the Fédération Internationale de Gynécologie et d'Obstétrique.[ |
|
I | Tumor limited to uterus. |
–IA | Tumor limited to endometrium/endocervix with no myometrial invasion. |
–IB | Less than or equal to half myometrial invasion. |
–IC | More than half myometrial invasion. |
II | Tumor extends to the pelvis. |
–IIA | Adnexal involvement. |
–IIB | Tumor extends to extrauterine pelvic tissue. |
III | Tumor invades abdominal tissues (not just protruding into the abdomen). |
–IIIA | One site. |
–IIIB | More than one site. |
–IIIC | Metastasis to pelvic and/or para-aortic lymph nodes. |
IV | |
–IVA | Tumor invades bladder and/or rectum. |
–IVB | Distant metastasis. |
References:
Surgery is often the principal means of diagnosis and is the primary treatment for all patients with uterine sarcoma. If the diagnosis is known, the extent of surgery is planned according to the stage of the tumor. Hysterectomy is usually performed when a uterine malignancy is suspected, except for rare instances when preservation of the uterus in a young patient is deemed safe for the type of cancer (e.g., a totally confined low-grade leiomyosarcoma in a woman who desires to retain childbearing potential). Medically suitable patients with the preoperative diagnosis of uterine sarcoma are considered candidates for abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic and periaortic selective lymphadenectomy. Cytologic washings are obtained from the pelvis and abdomen. Thorough examination of the diaphragm, omentum, and upper abdomen is performed.
There is no firm evidence from a prospective study that adjuvant chemotherapy or radiation therapy is of benefit for patients with uterine sarcoma.[
References:
Treatment Options for Stage I Uterine Sarcoma
Treatment options for stage I uterine sarcoma include:
A nonrandomized Gynecologic Oncology Group study examined the effect of pelvic radiation therapy on patients with stage I and II carcinosarcomas. Patients who had pelvic radiation therapy had a significant reduction in tumor recurrences within the radiation treatment field but no alteration in survival.[
Current Clinical Trials
Use our
References:
Treatment Options for Stage II Uterine Sarcoma
Treatment options for stage II uterine sarcoma include:
A nonrandomized Gynecologic Oncology Group study examined the effect of pelvic radiation therapy on patients with stage I and II carcinosarcomas. Patients who had pelvic radiation therapy had a significant reduction in tumor recurrences within the radiation treatment field but no alteration in survival.[
Current Clinical Trials
Use our
References:
Treatment Options for Stage III Uterine Sarcoma
Treatment options for stage III uterine sarcoma include:
Phase II chemotherapy studies by the Gynecologic Oncology Group have documented some antitumor activity for cisplatin, doxorubicin, and ifosfamide.[
GOG-108 was a randomized trial that examined the use of ifosfamide with or without cisplatin as first-line therapy for patients with measurable advanced or recurrent carcinosarcomas. Patients in the combination arm had a higher response rate (54% vs. 34%) and longer progression-free survival (PFS) (6 months vs. 4 months). However, patients did not have a significant improvement in survival (9 months vs. 8 months).[
A role for chemotherapy as an adjuvant to surgery has not been established.
Current Clinical Trials
Use our
References:
Treatment Options for Stage IV Uterine Sarcoma
There is currently no standard therapy for patients with stage IV disease. These patients should enroll in an ongoing clinical trial.
Phase II chemotherapy studies by the Gynecologic Oncology Group have documented some antitumor activity for cisplatin, doxorubicin, and ifosfamide.[
GOG-108 was a randomized trial that examined the use of ifosfamide with or without cisplatin as first-line therapy for patients with measurable advanced or recurrent carcinosarcomas. Patients in the combination arm had a higher response rate (54% vs. 34%) and longer progression-free survival (PFS) (6 months vs. 4 months). However, patients did not have a significant improvement in survival (9 months vs. 8 months).[
Current Clinical Trials
Use our
References:
Treatment Options for Recurrent Uterine Sarcoma
There is currently no standard therapy for patients with recurrent disease. These patients should enroll in an ongoing clinical trial.
Phase II chemotherapy studies by the Gynecologic Oncology Group have documented some antitumor activity for cisplatin, doxorubicin, and ifosfamide.[
GOG-108 was a randomized trial that examined the use of ifosfamide with or without cisplatin as first-line therapy for patients with measurable advanced or recurrent carcinosarcomas. Patients in the combination arm had a higher response rate (54% vs. 34%) and longer progression-free survival (PFS) (6 months vs. 4 months). However, patients did not have a significant improvement in survival (9 months vs. 8 months).[
Radiation therapy may be an effective method of palliative care for patients with carcinosarcoma who have localized recurrence in the pelvis confirmed by computed tomography. Phase I and II clinical trials are appropriate for patients who have disease recurrence with distant metastasis and are unresponsive to first-line phase II trials. High-dose progesterone hormone therapy may be of some benefit to patients with low-grade stromal sarcoma.[
Current Clinical Trials
Use our
References:
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary.
This summary is written and maintained by the
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of uterine sarcoma. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.
Reviewers and Updates
This summary is reviewed regularly and updated as necessary by the
Board members review recently published articles each month to determine whether an article should:
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewer for Uterine Sarcoma Treatment is:
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's
Levels of Evidence
Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a
Permission to Use This Summary
PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as "NCI's PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary]."
The preferred citation for this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Uterine Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at:
Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in
Disclaimer
Based on the strength of the available evidence, treatment options may be described as either "standard" or "under clinical evaluation." These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the
Contact Us
More information about contacting us or receiving help with the Cancer.gov website can be found on our
Last Revised: 2024-12-17
This information does not replace the advice of a doctor. Ignite Healthwise, LLC, disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the
Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Ignite Healthwise, LLC.
Individual and family medical and dental insurance plans are insured by Cigna Health and Life Insurance Company (CHLIC), Cigna HealthCare of Arizona, Inc., Cigna HealthCare of Illinois, Inc., Cigna HealthCare of Georgia, Inc., Cigna HealthCare of North Carolina, Inc., Cigna HealthCare of South Carolina, Inc., and Cigna HealthCare of Texas, Inc. Group health insurance and health benefit plans are insured or administered by CHLIC, Connecticut General Life Insurance Company (CGLIC), or their affiliates (see
All insurance policies and group benefit plans contain exclusions and limitations. For availability, costs and complete details of coverage, contact a licensed agent or Cigna sales representative. This website is not intended for residents of New Mexico.