Uterine sarcoma
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Overview
A uterine sarcoma is a malignant tumor that arises from the smooth muscle or connective tissue of the uterus. If the lesion originates from the stroma of the uterine lining it is an endometrial stromal sarcoma, and if the uterine muscle cell is the originator the tumor is a uterine leiomyosarcoma. A lesion that also contains malignant tumor cells of epithelial origin is termed uterine carcinosarcoma (formerly called malignant mixed mesodermal/mullerian tumor).
Classification
- Cellular Classification of Uterine Sarcoma.
- The most common histologic types of uterine sarcomas include:
- Carcinosarcomas (mixed mesodermal sarcomas [40%–50%]).
- Leiomyosarcomas (30%).
- Endometrial stromal sarcomas (15%).
The uterine neoplasm classification of the International Society of Gynecologic Pathologists and the World Health Organization uses the term carcinosarcomas for all primary uterine neoplasms containing malignant elements of both epithelial and stromal light microscopic appearances, regardless of whether malignant heterologous elements are present.
Histology
Tumoral entities include leiomyosarcomas, endometrial stromal sarcomas, carcinosarcomas and "other" sarcomas.[1]
- If the lesion originates from the stroma of the uterine lining it is an endometrial stromal sarcoma.
- If the uterine muscle cell is the originator the tumor is a uterine leiomyosarcoma.
- Carcinosarcomas comprise both malignant epithelial and malignant sarcomatous components.
Epidemiology
The vast majority of malignancies of the uterine body are endometrial carcinomas - only about 4% will be uterine sarcomas.[2]
Risk Factors
- The only documented etiologic factor in 10% to 25% of these malignancies is prior pelvic radiation therapy, which is often administered for benign uterine bleeding that began 5 to 25 years earlier.
- An increased incidence of uterine sarcoma has been associated with tamoxifen in the treatment of breast cancer.[3]
Signs and Symptoms
- Unusual or postmenopausal bleeding may be a sign of a malignancy including uterine sarcoma and needs to be investigated
- Pelvic pain
- Pelvic pressure
- Unusual vaginal discharge
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.
Staging
Uterine sarcoma is staged like endometrial carcinoma at time of surgery using the FIGO cancer staging system.
- Stage IA: tumor is limited to the endometrium
- Stage IB: invasion of less than half the myometrium
- Stage IC: invasion of more than half the myometrium
- Stage IIA: endocervical glandular involvement only
- Stage IIB: cervical stromal invasion
- Stage IIIA: tumor invades serosa or adnexa, or malignant peritoneal cytology
- Stage IIIB: vaginal metastasis
- Stage IIIC: metastasis to pelvic or para-aortic lymph nodes
- Stage IVA: invasion of the bladder or bowel
- Stage IVB: distant metastasis, including intraabdominal or inguinal lymph nodes
Therapy
Surgery is often the principal means of diagnosis and is the primary treatment for all patients with uterine sarcoma. If the diagnosis is known, the extent of surgery is planned according to the stage of the tumor. Stage I Uterine Sarcoma Standard treatment options: Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic and periaortic selective lymphadenectomy). Surgery plus pelvic radiation therapy. Surgery plus adjuvant chemotherapy. Surgery plus adjuvant radiation therapy as seen in the EORTC-55874 trial, for example.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.
Stage II Uterine Sarcoma Standard treatment options: Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic and periaortic selective lymphadenectomy). Surgery plus pelvic radiation therapy. Surgery plus adjuvant chemotherapy. Surgery plus adjuvant radiation therapy (EORTC-55874).
See also
References
- ↑ Zagouri F, Dimopoulos AM, Fotiou S, Kouloulias V, Papadimitriou CA (2009). "Treatment of early uterine sarcomas: disentangling adjuvant modalities". World J Surg Oncol. 7: 38. doi:10.1186/1477-7819-7-38. PMC 2674046. PMID 19356236.
- ↑ [1] American Cancer Society information, accessed 03-11-2006
- ↑ "uterine sarcoma".
External links
- Uni-Bonn site with detailed information, accessed 03-11-2006