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Uterine Sarcomas


Pedro T. Ramirez

, MD, Houston Methodist Hospital;

Gloria Salvo

, MD, MD Anderson Cancer Center

Reviewed/Revised Sep 2023
Topic Resources

Uterine sarcomas are a group of disparate, aggressive cancers of the uterine corpus. Common manifestations include abnormal uterine bleeding and pelvic pain or mass. For suspected uterine sarcoma, endometrial biopsy or dilation and curettage may be done, but results are often falsely negative; most sarcomas are diagnosed histologically after hysterectomy or myomectomy. Treatment requires total abdominal hysterectomy and bilateral salpingo-oophorectomy; for advanced cancer, chemotherapy and sometimes radiation therapy are indicated.

Uterine sarcomas are rare, accounting for 2 to 5% of all uterine cancers (1 General reference Uterine sarcomas are a group of disparate, aggressive cancers of the uterine corpus. Common manifestations include abnormal uterine bleeding and pelvic pain or mass. For suspected uterine sarcoma... read more ). Leiomyosarcoma and endometrial stromal sarcoma are the most common types. Average age at diagnosis is 60 years old. Uterine leiomyosarcoma incidence in the United States is 2 times higher in Black women than in White women.

Risk factors for uterine sarcomas are

  • Prior pelvic radiation

  • Tamoxifen use

Uterine sarcomas include

  • Leiomyosarcoma (the most common subtype [63%])

  • Endometrial stromal sarcoma (21%)

  • Undifferentiated uterine sarcoma

Rare uterine mesenchymal sarcoma subtypes include

  • Adenosarcomas

  • Perivascular epithelioid cell tumor (PEComas)

  • Rhabdomyosarcoma

High-grade uterine sarcomas tend metastasize hematogenously, most often to the lungs; lymph node metastases are uncommon.

General reference

Symptoms and Signs of Uterine Sarcomas

Most sarcomas manifest as abnormal uterine bleeding and, less commonly, as pelvic pain, a feeling of fullness in the abdomen, a mass in the vagina, frequent urination, or a palpable pelvic mass.

Diagnosis of Uterine Sarcomas

  • Histology, most often after surgical removal

Symptoms suggesting uterine sarcoma usually prompt transvaginal ultrasonography and endometrial biopsy or dilation and curettage (D & C). However, these tests have limited sensitivity for sarcoma. Endometrial stromal sarcoma and uterine leiomyosarcoma are often incidentally diagnosed histologically after hysterectomy or myomectomy.

If cancer is identified preoperatively, CT or MRI is typically done. If uterine sarcoma is diagnosed after surgical removal, imaging is recommended, and surgical re-exploration can be considered to complete staging.

Screening for Lynch syndrome (hereditary nonpolyposis colorectal cancer) is not usually done when patients have uterine sarcoma because this genetic syndrome increases risk of endometrial carcinoma, not sarcoma.


Staging is done surgically (see tables and ).


Treatment of Uterine Sarcomas

  • Total abdominal hysterectomy and bilateral salpingo-oophorectomy

  • Usually adjuvant pelvic radiation therapy

  • Chemotherapy for advanced or recurrent cancers

(See also National Comprehensive Cancer Network (NCCN): NCCN Clinical Practice Guidelines in Oncology: Uterine Sarcomas.)

Treatment of uterine sarcomas is total abdominal hysterectomy and bilateral salpingo-oophorectomy.

Uterine sarcomas should be removed en bloc; morcellation is contraindicated. If a specimen is fragmented during surgery, imaging is recommended to check for metastases, and re-exploration can be considered. Treatment with chemotherapy is also recommended.

The ovaries may be preserved in certain patients with early-stage uterine leiomyosarcoma if they wish to retain hormonal function. Additional surgical resection should be based on intraoperative findings.

For inoperable sarcomas, pelvic radiation therapy with or without brachytherapy and/or systemic therapy is recommended.

Adjuvant radiation therapy is typically used and appears to delay local recurrence but does not improve overall survival rate.

Chemotherapy agents are typically used when tumors are advanced or recur; drugs vary by tumor type.

Some preferred regimens are

  • Doxorubicin

  • Docetaxel/gemcitabine (preferred for leiomyosarcoma)

Overall, response to chemotherapy is poor.

Metastatic or unresectable uterine leiomyosarcomas have a poor prognosis. A multicenter randomized open-label superiority phase III trial in patients with metastatic or unresectable leiomyosarcomas showed that first-line therapy with doxorubicin plus trabectedin compared with doxorubicin alone resulted in significantly increased median progression-free survival (12.2 versus 6.2 months; adjusted hazard ratio, 0·41) (3 Treatment references Uterine sarcomas are a group of disparate, aggressive cancers of the uterine corpus. Common manifestations include abnormal uterine bleeding and pelvic pain or mass. For suspected uterine sarcoma... read more ).

Endocrine therapy is used for patients with endometrial stromal sarcoma or hormone receptor–positive uterine leiomyosarcoma. Progestins are frequently effective. Endocrine therapy may include:

  • Medroxyprogesterone acetate

  • Megestrol acetate

  • Aromatase inhibitors

  • GnRH (gonadotropin-releasing hormone) agonists

Treatment references

  • 1. Smith ES, Jansen C, Miller KM, et al: Primary characteristics and outcomes of newly diagnosed low-grade endometrial stromal sarcoma. Int J Gynecol Cancer 32(7):882-890, 2022. Published 2022 Jul 4. doi:10.1136/ijgc-2022-003383

  • 2. Leitao MM, Sonoda Y, Brennan MF, et al: Incidence of lymph node and ovarian metastases in leiomyosarcoma of the uterus. Gynecol Oncol 91(1):209-212, 2003. doi:10.1016/s0090-8258(03)00478-5

  • 3. Pautier P, Italiano A, Piperno-Neumann S, et al: Doxorubicin alone versus doxorubicin with trabectedin followed by trabectedin alone as first-line therapy for metastatic or unresectable leiomyosarcoma (LMS-04): a randomised, multicentre, open-label phase 3 trial. Lancet Oncol 23(8):1044-1054, 2022. doi:10.1016/S1470-2045(22)00380-1

Prognosis for Uterine Sarcomas

Prognosis for patients with uterine sarcomas is generally poorer than that with endometrial carcinoma of similar stage; survival is generally poor when the cancer has spread beyond the uterus.

  • Stage I: 51%

  • Stage II: 13%

  • Stage III: 10%

  • Stage IV: 3%

Most commonly, the cancer recurs locally, in the abdomen, or the lungs.

Prognosis reference

Key Points

  • Uterine sarcomas are uncommon.

  • Symptoms include abnormal vaginal bleeding, a mass in the vagina, pelvic pain, a feeling of fullness in the abdomen, and frequent urination.

  • Prognosis is generally worse than that with endometrial carcinoma of similar stage.

  • Treat most patients with total abdominal hysterectomy, bilateral salpingo-oophorectomy, and adjuvant radiation.

  • Treat patients who have endometrial stroma sarcoma and hormone receptor–positive leiomyosarcomas with endocrine therapy.

  • Treat inoperable sarcomas with radiation therapy and/or chemotherapy.

More Information

The following English-language resource may be useful. Please note that THE MANUAL is not responsible for the content of this resource.

  • National Cancer Institute: Uterine Cancer: This web site provides links to information about genetics and treatment of uterine sarcomas (a type of uterine cancer), as well as links to statistics and supportive and palliative care.

Drugs Mentioned In This Article

Drug Name Select Trade
Nolvadex, Soltamox
Adriamycin, Adriamycin PFS, Adriamycin RDF, Rubex
Docefrez , Taxotere
Gemzar, Infugem
Amen, Depo-Provera, Depo-subQ Provera 104, Provera
Megace, Megace ES
NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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