Uterine sarcomas are a group of disparate, highly malignant cancers developing from the uterine corpus.

Sarcomas account for < 5% of uterine cancers. Risk factors are similar to those for endometrial carcinoma (see Gynecologic Tumors: Endometrial Cancer). The most common types are mixed mesodermal tumors (malignant mixed müllerian tumor, in which the sarcoma is mixed with adenocarcinoma; recently renamed carcinosarcoma), leiomyosarcomas, and endometrial stromal tumors.

Symptoms and Signs

Most sarcomas manifest as abnormal vaginal bleeding and, less commonly, as pelvic pain or a palpable pelvic mass.

Diagnosis

• Transvaginal ultrasonography and endometrial biopsy or fractional D & C

Symptoms usually prompt transvaginal ultrasonography and endometrial biopsy or fractional D & C. If cancer is identified, CT or MRI is typically done preoperatively.

Staging is done surgically as follows:

• Stage I: Confined to the corpus
• Stage II: Confined to the corpus and cervix
• Stage III: Spread outside the uterus but confined to the pelvis
• Stage IV: Spread outside the true pelvis or into the mucosa of the bladder or rectum

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 51% for stage I, 13% for stage II, 10% for stage III, and 3% for stage IV. Most commonly, the cancer recurs locally, in the abdomen, and the lungs.

Treatment

• Total abdominal hysterectomy, bilateral salpingo-oophorectomy, and complete exploration of the abdomen

Treatment is total abdominal hysterectomy and bilateral salpingo-oophorectomy with complete exploration of the abdomen. Pelvic and para-aortic nodes are dissected in patients with carcinosarcoma. The usefulness of lymphadenectomy in patients with leiomyosarcoma or endometrial stromal sarcoma is controversial; no therapeutic value has been shown.

Adjuvant radiation therapy is typically used and appears to delay local recurrence but does not improve overall survival rate. Chemotherapy drugs vary with tumor type. Overall, response to chemotherapy is poor, although progestins are frequently effective for endometrial stromal tumors.

Last full review/revision November 2008 by David M. Gershenson, MD; Pedro T. Ramirez, MD

Content last modified February 2012