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Uterine Sarcomas


Pedro T. Ramirez

, MD, The University of Texas MD Anderson Cancer Center;

Gloria Salvo

, MD, MD Anderson Cancer Center

Reviewed/Revised Jul 2022 | Modified Sep 2022
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Uterine sarcomas are a group of disparate, highly malignant cancers developing from the uterine corpus. Common manifestations include abnormal uterine bleeding and pelvic pain or mass. For suspected uterine sarcoma, endometrial biopsy or dilation and curettage can 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.

In the US, uterine sarcoma is rare; 5058 cases of uterine sarcomas were estimated to occur in 2018. Uterine sarcomas account for about 3 to 7% of all uterine cancers (1 General reference Uterine sarcomas are a group of disparate, highly malignant cancers developing from the uterine corpus. Common manifestations include abnormal uterine bleeding and pelvic pain or mass. For suspected... read more ).

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.



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.

In one study, 5-year survival rates were

  • Stage I: 51%

  • Stage II: 13%

  • Stage III: 10%

  • Stage IV: 3%

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

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.

Lymphadenectomy in patients with leiomyosarcoma or endometrial stromal sarcoma is not indicated because there is evidence that the risk of lymph node metastases is minimal (< 2%).

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

Preferred regimens are

  • Doxorubicin

  • Docetaxel/gemcitabine (preferred for leiomyosarcoma)

Other recommended regimens include

  • Doxorubicin/ifosfamide

  • Doxorubicin/dacarbazine

  • Gemcitabine/dacarbazine

  • Gemcitabine/vinorelbine

  • Dacarbazine

  • Gemcitabine

  • Epirubicin

  • Ifosfamide

  • Liposomal doxorubicin

  • Pazopanib

  • Temozolomide

  • Trabectedin

  • Eribulin

Overall, response to chemotherapy is poor.

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

  • Medroxyprogesterone acetate

  • Megestrol acetate

  • Aromatase inhibitors

  • GnRH (gonadotropin-releasing hormone) agonists

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 is an English-language resource that 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
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