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Uterine Sarcomas

By Pedro T. Ramirez, MD, Professor, Department of Gynecologic Oncology and Reproductive Medicine, David M. Gershenson Distinguished Professor in Ovarian Cancer Research, and Director of Minimally Invasive Surgical Research and Education, The University of Texas MD Anderson Cancer Center ; David M. Gershenson, MD, Professor and Chairman, Department of Gynecologic Oncology and Reproductive Medicine, The University of Texas MD Anderson Cancer Center ; Gloria Salvo, MD, Rotating Research Resident, Department of Gynecologic Oncology and Reproductive Medicine, MD Anderson Cancer Center

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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 D & C 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, an estimated 4910 cases of uterine sarcomas are will occur in 2017. Uterine sarcomas account for about 3% 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 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

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. 7. New York, Springer, 2010. (See also National Cancer Institute: Uterine Sarcoma Treatment.)

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. 7. New York, Springer, 2010. (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. Histology is not an independent prognostic factor.

In one study, 5-yr 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

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.

The usefulness of lymphadenectomy in patients with leiomyosarcoma or endometrial stromal sarcoma is controversial; no therapeutic value has been shown.

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.

  • Most sarcomas are asymptomatic; 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.

Resources In This Article

Drugs Mentioned In This Article

  • Drug Name
    Select Trade
  • IFEX
  • NOLVADEX
  • NAVELBINE
  • TAXOTERE
  • MEGACE
  • GEMZAR
  • DTIC-DOME
  • PROVERA

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