Uterine sarcoma

The uterine sarcomas form a group of malignant tumors that arises from the smooth muscle or connective tissue of the uterus.

Histology

Tumoral entities include leiomyosarcomas, endometrial stromal sarcomas, carcinosarcomas and "other" sarcomas.[1]

Classification

Leiomyosarcomas are now staged using the 2009 FIGO staging system[2] (previously they were staged like endometrial carcinomas) at time of surgery.

IA: ≤5 cm in greatest dimension
IB: >5 cm
IIA: involves adnexa of uterus
IIB: involves other pelvic tissues
IIIA: 1 site
IIIB: >1 site
IIIC: regional lymph node metastasis

Endometrial stromal sarcomas and uterine adenosarcomas are classified as above, with the exception of different classifications for Stage I tumors.

IA: limited to endometrium/endocervix
IB: invades <½ myometrium
IC: invades ≥½ myometrium

Finally, malignant mixed Müllerian tumors, a type of carcinosarcoma, are staged similarly to endometrial carcinomas.[3]

IA: invades <½ myometrium
IB: invades ≥½ myometrium
IIIA: invades uterine serosa and/or adnexa
IIIB: vaginal and/or parametrial involvement
IIIC: metastases to pelvic and/or paraaortic lymph nodes
IIIC1: positive pelvic nodes
IIIC2: positive para-aortic lymph nodes

Signs and Symptoms

Unusual or postmenopausal bleeding may be a sign of a malignancy including uterine sarcoma and needs to be investigated. Other signs include pelvic pain, pressure, and unusual discharge. A nonpregnant uterus that enlarges quickly is suspicious. However, none of the signs are specific. Specific screening test have not been developed; a Pap smear is a screening test for cervical cancer and not designed to detect uterine sarcoma.

Diagnosis

Investigations by the physician include imaging (ultrasound, CAT scan, MRI) and, if possible, obtaining a tissue diagnosis by biopsy, hysteroscopy, or D&C. Ultimately the diagnosis is established by the histologic examination of the specimen. Typically malignant lesions have >10 mitosis per high power field. In contrast a uterine leiomyoma as a benign lesion would have < 5 mitosis per high power field.

Management

Therapy is based on staging and patient condition and utilizes one or more of the following approaches. Surgery is the mainstay of therapy if feasible involving total abdominal hysterectomy with bilateral salpingo-oophorectomy. Other approaches include radiation therapy, chemotherapy, and hormonal therapy.

Prognosis is relatively poor.[4]

Epidemiology

Uterine sarcoma are rare, out of all malignancies of the uterine body only about 4% will be uterine sarcomas.[5] Generally, the cause of the lesion is not known, however patients with a history of pelvic radiation are at higher risk. Most tumors occur after menopause. Women who take long-term tamoxifen are at higher risk.[6]

See also

References

External links

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