Sarcoma (Uterine)

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Chapter 119 Sarcoma (Uterine)

INTRODUCTION

Description: Uterine sarcoma is characterized by sarcomatous change in the tissues of the Müllerian system including the endometrial stroma and myometrium. Mixed Müllerian sarcomas may include elements not native to the genital tract such as cartilage or bone (heterologous type).

Prevalence: Less than 5% of uterine malignancies, 1 of 800 smooth muscle tumors, 0.67 of 100,000 women older than age 20.

Predominant Age: 40 to 60, mean age 52.

Genetics: No genetic pattern. Seen more often in African-American women, although there is no racial predisposition.

ETIOLOGY AND PATHOGENESIS

Causes: Etiology unknown.

Risk Factors: Leiomyomata, estrogen and obesity have also been proposed; oral contraceptive use is associated with a reduced risk.

CLINICAL CHARACTERISTICS

Signs and Symptoms

• Bleeding and passage of tissue

• Lower abdominal pain and mass

• Rapid enlargement of the uterus

• Uterine growth after menopause

DIAGNOSTIC APPROACH

Differential Diagnosis

• Benign leiomyomata

• Cervical cancer

• Ovarian cancer metastatic to the endometrium

• Metachronous Müllerian tumor

Associated Conditions: Breast or colon cancer.

Workup and Evaluation

Laboratory: No evaluation indicated except for preoperative screening.

Imaging: Chest radiograph (for metastases), transvaginal ultrasonography, or sonohysterography may be useful.

Special Tests: None. (Endometrial biopsy results are rarely positive.)

Diagnostic Procedures: Diagnosis rarely made before surgery.

Pathologic Findings

Based on cell type and tissue involved. Most often soft fleshy tumors with a gray–yellow or pink character. Areas of necrosis and hemorrhage are common (75%). Vascular invasion is present in 10% to 20% of patients. Higher mitotic rates are associated with greater atypia.

MANAGEMENT AND THERAPY

Nonpharmacologic

General Measures: Evaluation and staging.

Specific Measures: Surgical exploration with hysterectomy, bilateral salpingo-oophorectomy, cytologic examination of the abdomen and diaphragm, and para-aortic node sampling. Radiation to the vaginal cuff reduces local recurrence (although objective evidence of improved survival is lacking).

Diet: No specific dietary changes indicated except as dictated by surgical therapy.

Activity: No restriction except as dictated by surgical therapy.

Patient Education: American College of Obstetricians and Gynecologists Patient Education Pamphlet AP097 (Cancer of the Uterus), AP008 (Hysterectomy), AP080 (Preparing for Surgery), AP095 (Abnormal Uterine Bleeding), AP074 (Uterine Fibroids).

Drug(s) of Choice

None. (Adjuvant chemotherapy [vincristine, actinomycin D, and cyclophosphamide or doxorubicin (Adriamycin)] has been advocated but an improvement in prognosis has not been demonstrated.) Temozolomide (an imidazotetrazine derivative) has been explored as an additional option.

FOLLOW-UP

Patient Monitoring: Follow-up Pap tests from vaginal cuff every 3 months for 2 years, then every 6 months for 3 years, then yearly. Chest radiograph annually.

Prevention/Avoidance: None.

Possible Complications: Distant spread.

Expected Outcome: Depends on stage of tumor and number of mitotic figures present. Overall survival is approximately 20% at 5 years; patients with stage I and II disease have survival rates of up to 40%.

MISCELLANEOUS

Pregnancy Considerations: Generally not a consideration because they are unlikely to coexist.

ICD-9-CM Codes: Specific to cell type and location.

Aaro LA, Symmonds RE, Dockerty MB. Sarcoma of the uterus. A clinical and pathologic study of 177 cases. Am J Obstet Gynecol. 1966;94:101.

Barter JF, Smith EB, Szpak CA, et al. Leiomyosarcoma of the uterus: clinicopathologic study of 21 cases. Gynecol Oncol. 1985;21:220.

Gallup DG, Cordray DR. Leiomyosarcoma of the uterus: case report and a review. Obstet Gynecol Surv. 1979;34:300.

Shakfeh AM, Woodruff JD. Primary ovarian sarcomas: report of 46 cases and review of literature. Obstet Gynecol. 1987;42:331.

Talbot SM, Keohan ML, Hesdorffer M, et al. A phase II trial of temozolomide in patients with unresectable or metastatic soft tissue sarcoma. Cancer. 2003;98:1942.

Kempson RL, Bari W. Uterine sarcomas. Classification, diagnosis, and prognosis. Hum Pathol. 1970;1:331.

Lu K, Slomivitz BM. Neoplastic diseases of the uterus. In: Katz VL, Lentz GM, Lobo RA, Gershenson DM, editors. Comprehensive Gynecology. 5th ed. Philadelphia: Mosby/Elsevier; 2007:829.

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