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Uterine Sarcomas

By

Pedro T. Ramirez

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


Gloria Salvo

, MD, MD Anderson Cancer Center

Last full review/revision Sep 2020| Content last modified Sep 2020
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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 8% of all uterine cancers.

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

Carcinosarcomas used to be categorized as sarcomas but are now considered and treated as high-grade epithelial tumors (carcinomas).

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

Symptoms and Signs

Most sarcomas manifest as abnormal vaginal 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

  • Histology, most often after surgical removal

Symptoms suggesting uterine sarcoma usually prompt transvaginal ultrasonography and endometrial biopsy or fractional dilation and curettage (D & C). However, these tests have limited sensitivity. 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.

Screening for hereditary nonpolyposis colorectal cancer (Lynch syndrome) is not usually done when patients have uterine sarcoma; in contrast, such screening is done when patients have endometrial cancer.

Staging

Table
icon

FIGO Surgical Staging of Uterine Sarcoma: Leiomyosarcoma and Endometrial Stromal Sarcoma

Stage

Description

I

Limited to the uterus

  • IA

Tumor ≤ 5 cm in largest dimension

  • IB

Tumor > 5 cm

II

Extending beyond the uterus but within the pelvis

  • IIA

Involving the adnexa

  • IIB

Involving other pelvic tissues

III

Infiltrating abdominal tissues

  • IIIA

In one site

  • IIIB

> 1 site

  • IIIC

Regional lymph node metastasis

IVA

Invading bladder or rectum

IVB

Distant metastases

Adapted from staging established by the International Federation of Gynecology and Obstetrics (FIGO) and American Joint Committee on Cancer (AJCC), AJCC Cancer Staging Manual, ed. 8. New York, Springer, 2017. (See also National Cancer Institute: Uterine Sarcoma Treatment.)

Table
icon

FIGO Surgical Staging of Uterine Sarcoma: Adenosarcoma

Stage

Description

I

Limited to the uterus

  • IA

Limited to the endometrium and/or endocervix

  • IB

Invading less than half the myometrium

  • IC

Invading more than half the myometrium

II

Extending beyond the uterus but within the pelvis

  • IIA

Involving the adnexa

  • IIB

Involving other pelvic tissues

III

Infiltrating abdominal tissues

  • IIIA

In one site

  • IIIB

> 1 site

  • IIIC

Metastasis to regional lymph nodes

IVA

Invading the bladder or rectum

IVB

Distant metastases

* Based on staging established by the International Federation of Gynecology and Obstetrics (FIGO) and American Joint Committee on Cancer (AJCC), AJCC Cancer Staging Manual, ed. 8. New York, Springer, 2017. (See also National Cancer Institute: Uterine Sarcoma Treatment.)

Prognosis

Prognosis is generally poorer than that with endometrial cancer 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

  • Total abdominal hysterectomy and bilateral salpingo-oophorectomy

(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, 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.

Combination chemotherapy regimens are recommended:

  • Docetaxel/gemcitabine (preferred for leiomyosarcoma)

  • Doxorubicin/ifosfamide

  • Doxorubicin/dacarbazine

  • Gemcitabine/dacarbazine

  • Gemcitabine/vinorelbine

Overall, response to chemotherapy is poor.

Hormone therapy is used for patients with endometrial stromal sarcoma or hormone receptor–positive uterine leiomyosarcoma. Progestins are frequently effective. Hormone 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 cancer of similar stage.

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

  • Use hormone therapy for patients with endometrial stroma sarcoma and hormone receptor–positive leiomyosarcomas.

  • 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
PROVERA
NAVELBINE
GEMZAR
DTIC-DOME
IFEX
NOLVADEX
TAXOTERE
MEGACE
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