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 of Uterine Sarcomas
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 of Uterine Sarcomas
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
Staging is done surgically (see tables FIGO Surgical Staging of Uterine Sarcoma: Leiomyosarcoma and Endometrial Stromal Sarcoma FIGO Surgical Staging of Uterine Sarcoma: Leiomyosarcoma and Endometrial Stromal Sarcoma 
and FIGO Surgical Staging of Uterine Sarcoma: Adenosarcoma FIGO Surgical Staging of Uterine Sarcoma: Adenosarcoma ).
Prognosis for Uterine Sarcomas
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 of Uterine Sarcomas
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 |
|---|---|
tamoxifen |
SOLTAMOX |
docetaxel |
TAXOTERE |
gemcitabine |
GEMZAR |
ifosfamide |
IFEX |
dacarbazine |
DTIC-DOME |
vinorelbine |
NAVELBINE |
medroxyprogesterone |
PROVERA |
megestrol |
MEGACE |
